C0NI£NIS

Pieface xxiii
Acknowledgment xxiv
Intioduction xxv
Approach to Dermatologic Diagnosis xxvi
Outline of Dermatologic Diagnosis xxvi
Special Clinical and Laboratory Aids to Dermatologic Diagnosis xxxv
PAkI I
DISCRDERS PRESENTINC IN THE SKIN
AND MUCCUS MEMßRANES
SECI|0N 1
0IS0k0£kS 0F S£8AC£0üS AN0 AF0CkIN£ CIAN0S 2
Acne Vulgaris (Common Acne) and Cystic Acne 2
Rosacea 9
Perioral Dermatitis 14
Hidradenitis Suppurativa 16
SECI|0N 2
£CI£MA[0£kMAIIIIS 20
Contact Dermatitis 20
Irritant Contact Dermatitis 20
Acute Iiiitant Contact Deimatitis 21
Chionic Iiiitant Contact Deimatitis 22
Allergic Contact Dermatitis 26
Alleigic Contact Deimatitis Due to Plants 29
Systemic ACD 32
Aiiboine ACD 32
Atopic Dermatitis 34
Lichen Simplex Chronicus 42
Prurigo Nodularis 44
Dyshidrotic Eczematous Dermatitis 45
Nummular Eczema 46
Autosensitization Dermatitis 48
Seborrheic Dermatitis 48
Asteatotic Dermatitis 52
SECI|0N 5
FS0kIASIS 53
Psoriasis Vulgaris 53
Pustular Psoriasis 62
Palmoplantai Pustulosis 62
Geneialized Acute Pustulai Psoiiasis (von Zumbusch) 64
C0|IE|I' vììì
Psoriatic Erythroderma 67
Psoriatic Arthritis 67
Management of Psoriasis 67
SECI|0N 4
IChIh¥0S£S T2
Dominant Ichthyosis Vulgaris 72
X-Linked Ichthyosis 75
Lamellar Ichthyosis 78
Epidermolytic Hyperkeratosis 81
Ichthyosis in the Newborn 83
Collodion Baby 83
Hailequin Fetus 84
Syndromic Ichthyosis 84
Acquired Ichthyosis 84
Inherited Keratodermas of Palms and Soles 84
Diffuse Palmoplantai Keiatodeima 85
Punctate Palmoplantai Keiatodeima 85
Focal Palmoplantai Keiatodeima 86
SECI|0N 5
MISC£IIAN£0üS £FI0£kMAI 0IS0k0£kS 88
Acanthosis Nigricans 88
Darier Disease 90
Grover Disease 92
Pityriasis Rubra Pilaris 93
Disseminated Superncial Actinic Porokeratosis 96
SECI|0N 6
8üII0üS 0IS£AS£S 98
Hereditary Epidermolysis Bullosa 98
Familial Benign Pemphigus 105
Pemphigus 106
Bullous Pemphigoid 112
Cicatricial Pemphigoid 114
Pemphigoid Gestationis 115
Dermatitis Herpetiformis 116
Linear IgA Dermatosis 119
Epidermolysis Bullosa Acquisita 120
SECI|0N 1
MISC£IIAN£0üS INFIAMMAI0k¥ 0IS0k0£kS 122
Pityriasis Rosea 122
Parapsoriasis en Plaques 124
Small-Plaque Paiapsoiiasis (Digitate Deimatosis) 124
Laige-Plaque Paiapsoiiasis (Paiapsoiiasis en Plaques) 126
Lichen Planus 128
Granuloma Annulare 134
C0|IE|I' ìx
Morphea 136
Lichen Sclerosus et Atrophicus 142
Pigmented Purpuric Dermatoses 144
Pityriasis Lichenoides (Acute and Chronic) 146
Erythema Multiforme Syndrome 148
Erythema Nodosum Syndrome 152
Other Panniculitides 154
Pyoderma Gangrenosum 156
Sweet Syndrome 160
Granuloma Faciale 163
SECI|0N 8
S£v£k£ AN0 IIF£-Ihk£AI£NINC £küFII0NS IN Ih£ ACüI£I¥ III FAII£NI 164
Exfoliative Erythroderma Syndrome 164
Rashes in the Acutely Ill Febrile Patient 170
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis 173
SECI|0N 9
8£NICN N£0FIASMS AN0 h¥F£kFIASIAS 1T8
Disorders of Melanocytes 178
Acquiied Nevomelanocytic Nevi 178
Halo Nevomelanocytic Nevus 183
Blue Nevus 184
Nevus Spilus 186
Spitz Nevus 188
Mongolian Spot 189
Nevus of Ota 190
Vascular Tumors and Malformations 192
Vascular Tumors 193
Hemangioma of Infancy 193
Pyogenic Gianuloma 198
Glomus Tumoi 199
Angiosaicoma 200
Vascular Malformations 201
Capillaiy Malfoimations 201
Porì-Víne Sìaín 201
S¡íJer Àngíoma 202
Venous La|e 204
C|erry Àngíoma 205
Àngío|eraìoma 206
Lymphatic Malfoimation 208
Lym¡|angíoma 208
Capillaiy/Venous Malfoimations 209
Miscellaneous Cysts and Pseudocysts 211
Epideimoid Cyst 212
Tiichilemmal Cyst 212
Epideimal Inclusion Cyst 212
Milium 213
Digital Myxoid Cyst 214
C0|IE|I' x
Miscellaneous Benign Neoplasms and Hyperplasias 215
Seboiiheic Keiatosis 215
Beckei Nevus 219
Tiichoepithelioma 220
Syiingoma 220
Sebaceous Hypeiplasia 222
Nevus Sebaceous 222
Epideimal Nevus 222
Benign Dermal and Subcutaneous Neoplasms and Hyperplasias 224
Lipoma 224
Deimatofbioma 224
Hypeitiophic Scais and Keloids 227
Infantile Digital Fibiomatosis 230
Skin Tag 231
SECI|0N 10
Fh0I0S£NSIIIvII¥, Fh0I0-IN0üC£0 0IS0k0£kS,
AN0 0IS0k0£kS 8¥ I0NIIINC kA0IAII0N 232
Skin Reactions to Sunlight 232
Acute Sun Damage (Sunbuin) 235
Diug-/Chemical-Induced Photosensitivity 236
Phototoxic Diug-/Chemical-Induced Photosensitivity 238
Sysìemít P|oìoìoxít Dermaìíìís 240
To¡íta| P|oìoìoxít Demaìíìís 241
P|yìo¡|oìoJermaìíìís 242
Photoalleigic Diug-/Chemical-Induced Photosensitivity 244
Polymorphous Light Eruption 248
Solar Urticaria 250
Photoexacerbated Dermatoses 250
Metabolic Photosensitivity - The Porphyrias 252
Por¡|yría Cuìanea TarJa 252
Varíegaìe Por¡|yría 257
Eryì|ro¡oíeìít Proìo¡or¡|yría 259
Chronic Photodamage 262
Deimatoheliosis (°Photoaging") 262
Solai Lentigo 264
Chondiodeimatitis Nodulaiis Helicis 266
Actinic Keiatosis 267
Skin Reactions to Ionizing Radiation 270
Radiation Deimatitis 270
SECI|0N 11
Fk£CANC£k0üS I£SI0NS AN0 CüIAN£0üS CAkCIN0MAS 2T4
Epidermal Precancers and Cancers 274
Epithelial Piecanceious Lesions and SCCIS 274
Solai oi Actinic Keiatosis 275
Cutaneous Hoin 275
Arsenical Keratoses 276
Squamous Cell Carcinoma in Situ 276
Invasive Squamous Cell Carcinoma 280
C0|IE|I' xì
Keratoakanthoma 286
Basal Cell Carcinoma 287
Basal Cell Nevus Syndrome 294
Malignant Appendage Tumors 296
Merkel Cell Carcinoma 296
Dermatonbrosarcoma Protuberans 298
Atypical Fibrosarcoma 299
SECI|0N 12
M£IAN0MA Fk£CükS0kS AN0 FkIMAk¥ CüIAN£0üS M£IAN0MA 300
Precursors of Cutaneous Melanoma 300
Dysplastic Melanocytic Nevus 300
Congenital Nevomelanocytic Nevus 304
Cutaneous Melanoma 308
Clinical Piesentations of Melanoma 310
Me|anoma ín Síìu 311
Lenìígo Ma|ígna Me|anoma 312
Su¡erftía| S¡reaJíng Me|anoma 315
NoJu|ar Me|anoma 320
Desmo¡|asìít Me|anoma 323
Àtra| Lenìígínous Me|anoma 324
Àme|anoìít Me|anoma 326
Ma|ígnanì Me|anoma o[ ì|e Mutosa 327
Metastatic Melanoma 328
Staging of Melanoma 331
Prognosis of Melanoma 332
Management of Melanoma 332
SECI|0N 15
FICM£NIAk¥ 0IS0k0£kS 334
Vitiligo 335
Albinism 341
Oculocutaneous Albinism 341
Melasma 344
Pigmentary Changes Following Inßammation of the Skin 346
Hypeipigmentation 346
Hypopigmentation 349
PAkI II
DERMATCLCCY AND INTERNAL MEDICINE
SECTICN 14
IHE 5KIN 5ICN5 IN IMMUNE, AUIOIMMUNE,
AND kHEUMAIIC DI5OkDEk5 354
Systemic Amyloidosis 354
Systemic AL Amyloidosis 354
Systemic AA Amyloidosis 356
Localized Cutaneous Amyloidosis 356
C0|IE|I' xìì
Urticaria and Angioedema 358
Behçet Disease 366
Dermatomyositis 370
Livedo Reticularis 374
Sneddon Syndiome 376
Lupus Erythematosus 376
Systemic Lupus Eiythematosus 378
Cutaneous Lupus Eiythematosus 380
Àtuìe Cuìaneous Lu¡us Eryì|emaìosus 380
Su|atuìe Cuìaneous Lu¡us Eryì|emaìosus 383
C|ronít Cuìaneous Lu¡us Eryì|emaìosus 384
C|assít C|ronít DístoíJ LE 384
C|ronít Lu¡us Pannítu|íìís 388
Scleroderma 389
Scleiodeima-Like Conditions 393
Raynaud Phenomenon 394
Vasculitis 397
Hypeisensitivity Vasculitis 397
Schönlein-Henoch Puipuia 399
Polyaiteiitis Nodosa 400
Wegenei Gianulomatosis 402
Giant Cell Aiteiitis 405
Uiticaiial Vasculitis 407
Nodulai Vasculitis 408
Kawasaki Disease 410
Reactive Arthritis 414
Sarcoidosis 417
SECTICN 15
ENDOCkINE, MEIAßOLIC, NUIkIIIONAL, AND CENEIIC DI5EA5E5 420
Skin Diseases in Pregnancy 420
Cholestasis of Piegnancy 420
Pustulai Psoiiasis in Piegnancy 420
Pemphigoid Gestationis 420
Polymoiphic Eiuption of Piegnancy 422
Piuiigo of Piegnancy and Atopic Eiuption of Piegnancy 422
Skin Manifestations of Obesity 424
Diabetes Mellitus 424
Skin Diseases Associated with Diabetes Mellitus 424
Diabetic Bullae 425
°Diabetic Foot" and Diabetic Neuiopathy 426
Diabetic Deimopathy 427
Neciobiosis Lipoidica 428
Cushing Syndrome and Hypercorticism 430
Graves Disease and Hyperthyroidism 431
Hypothyroidism and Myxedema 431
Addison Disease 433
Metabolic and Nutritional Disorders 434
Xanthomas 434
C0|IE|I' xììì
Xanì|e|asma 436
Xanì|oma TenJíneum 436
Xanì|oma Tu|erosum 436
Eru¡ìí·e Xanì|oma 438
Xanì|oma Sìríaìum Pa|mare 438
Normo|í¡emít P|ane Xanì|oma 439
Scuivy 440
Zinc Defciency and Aciodeimatitis Enteiopathica 442
Pellagia 445
Gout 446
Genetic Diseases 448
Pseudoxanthoma Elasticum 448
Tubeious Scleiosis 449
Neuiofbiomatosis 453
Heieditaiy Hemoiihagic Telangiectasia 457
SECTICN 16
5KIN 5ICN5 OF VA5CULAk IN5UFFICIENCY 458
Atherosclerosis, Arterial Insufnciency, and Atheroembolization 458
Thromboangiitis Obliterans 462
Thrombophlebitis and Deep Venous Thrombosis 462
Chronic Venous Insufnciency 465
Most Common Leg/Foot Ulcers 471
Livedoid Vasculitis 475
Chronic Lymphatic Insufnciency 476
Pressure Ulcers 477
SECTICN 17
5KIN 5ICN5 OF kENAL IN5UFFICIENCY 480
Classincation of Skin Changes 480
Calciphylaxis 480
Nephrogenic Fibrosing Dermopathy 483
Acquired Perforating Disorders 485
SECTICN 18
5KIN 5ICN5 OF 5Y5IEMIC CANCEk5 486
Mucocutaneous Signs of Systemic Cancers 486
Classifcation of Skin Signs of Systemic Cancei 486
Metastatic Cancer to the Skin 487
Paget Disease 494
Mammaiy Paget Disease 494
Extiamammaiy Paget Disease 496
Cowden Syndrome (Multiple Hamartoma Syndrome) 498
Peutz-Jeghers Syndrome 498
Glucagonoma Syndrome 500
Malignant Acanthosis Nigricans 502
Paraneoplastic Pemphigus 503
C0|IE|I' xìv
SECTICN 19
5KIN 5ICN5 OF HEMAIOLOCIC DI5EA5E 504
Thrombocytopenic Purpura 504
Disseminated Intravascular Coagulation 506
Cryoglobulinemia 509
Leukemia Cutis 511
Langerhans Cell Histiocytosis 514
Mastocytosis Syndromes 519
SECTICN 20
CUIANEOU5 LYMPHOMA5 AND 5AkCOMA 524
Adult T Cell Leukemia/Lymphoma 524
Cutaneous T Cell Lymphoma 526
Mycosis Fungoides 526
Mycosis Fungoides Vaiiants 530
Sézaiy Syndiome 534
Lymphomatoid Papulosis 535
Cutaneous Anaplastic Large Cell Lymphomas 535
Cutaneous B Cell Lymphoma 537
Kaposi Sarcoma 538
SECTICN 21
5KIN DI5EA5E5 IN OkCAN AND ßONE MAkkOW IkAN5PLANIAIION 544
Most Common Infections Associated with Organ Transplantation 544
Skin Cancers Associated with Organ Transplantation 544
Graft-versus-Host Disease 546
Acute Cutaneous GVHR 546
Chionic Cutaneous GVHR 550
SECTICN 22
ADVEk5E CUIANEOU5 DkUC kEACIION5 552
Clinical Types of Adverse Cutaneous Drug Reactions 553
Exanthematous Drug Reactions 557
Pustular Eruptions 561
Drug-Induced Acute Urticaria, Angioedema, Edema, and Anaphylaxis 563
Fixed Drug Eruption 566
Drug Hypersensitivity Syndrome 568
Drug-Induced Pigmentation 570
Pseudoporphyria 574
ACDR-Related Necrosis 575
ACDR Related to Chemotherapy 579
C0|IE|I' xv
SECTICN 23
DI5OkDEk5 OF P5YCHIAIkIC EIIOLOCY 582
Classincation of Disorders of Psychiatric Etiology 582
Dysmorphic Syndrome 582
Delusions of Parasitosis 582
Neurotic Excoriations and Trichotillomania 583
Factitious Syndromes (Münchausen Syndrome) 586
Cutaneous Signs of Injecting Drug Use 587
PAkI III
DISEASES DUE TC MICRCßIAL ACENTS
SECI|0N 24
8ACI£kIAI INF£CII0NS INv0IvINC Ih£ SkIN 590
Stuphy|vcvccus uureus : Infections and Intoxications 591
Streptvcvccus Pyvgenes : Infections and Intoxications 591
Superncial Bacterial Epidermal Colonizations and Infections 592
Eiythiasma 592
Pitted Keiatolysis 594
Tiichomycosis 595
Nonspecifc Inteitiigo 596
Pyoderma 597
Impetigo and Ecthyma 597
Abscess, Fuiuncle, and Caibuncle 605
Soft Tissue Infections 609
Eiysipelas and Cellulitis 609
Neciotizing Soft Tissue Infections 618
Lymphangitis 619
Wound Infections 621
Gram-Positive Coccal Infections Associated with Toxin Production 625
(Intoxications)
Staphylococcal Scalded-Skin Syndiome 626
Toxic Shock Syndiome 629
Scailet Fevei 631
Gram-Positive Bacillary Infections Associated with Toxin Production 634
Cutaneous Anthiax 634
Cutaneous Diphtheiia 637
Tetanus 637
Infective Endocarditis, Sepsis, and Septic Shock 638
Infective Endocaiditis 638
Sepsis and Septic Shock 642
Neíssería 646
Neíssería meníngíìíJís Infection 646
Neíssería gonorr|oeae Infections 649
NeIsserIu gvnvrrhveue : Local Infections 650
Disseminated Gonococcal Infection 652
C0|IE|I' xvì
Gram-Negative Infections 654
Barìone||a Infections 654
Cat-Sciatch Disease 655
Barìone||a Infections in HIV/AIDS 658
Bacillaiy Angiomatosis 658
Tulaiemia 659
PseuJomonas Species 662
Cutaneous PseuJomonas aerugínosa Infections 662
Mycobacterial Infections 664
Classifcation of Mycobacteiia and Mycobacteiial Infections 665
Lepiosy 665
Cutaneous Tubeiculosis 671
Nontubeiculous Mycobacteiial Infections 677
Myto|atìeríum marínum Infection 678
Myto|atìeríum u|terans Infection 680
Myto|atìeríum [orìuíìum Complex Infections 682
Lyme Borreliosis 684
SECI|0N 25
FüNCAI INF£CII0NS 0F Ih£ SkIN AN0 hAIk 692
Superncial Fungal Infections 692
Dermatophytoses 693
Deimatophytoses of Epideimis 697
Tínea PeJís 697
Tínea Manuum 701
Tínea Crurís 703
Tínea Cor¡orís 704
Tínea Fatía|ís 707
Tínea Intogníìo 708
Deimatophytoses of Haii 708
Tínea Ca¡íìís 709
Tínea Bar|ae 715
Dermaìo¡|yìít Fo||ítu|íìís 716
Ma,ott|í Cranu|oma 717
Candidiasis 718
Cutaneous Candidiasis 720
Oiophaiyngeal Candidiasis 724
Genital Candidiasis 727
Candidiasis of Nail Appaiatus 730
Chionic Mucocutaneous Candidiasis 730
Mu|ussezIu Infections 732
Pityiiasis Veisicoloi 732
Ma|asse:ía Folliculitis 734
TrIchvspvrvn Infections 736
Tinea Nigra 736
Invasive and Disseminated Fungal Infections 737
Subcutaneous Mycoses 737
Myteìoma 738
C|romomytosís 742
S¡oroìrít|osís 744
C0|IE|I' xvìì
Systemic Fungal Infections with Dissemination to Skin 747
DíssemínaìeJ Cry¡ìotottosís 747
Hísìo¡|asmosís 749
B|asìomytosís : Cutaneous Manifestations 753
DíssemínaìeJ CottíJíoíJomytosís 755
DíssemínaìeJ Penítí||ínosís 758
Penítí||ínosís 758
Àtuìe CanJíJemía anJ DíssemínaìeJ CanJíJíasís 758
SECI|0N 26
kICk£IISIAI INF£CII0NS 760
Tick-Borne Spotted Fever 761
Rot|y Mounìaín S¡oììeJ Fe·er 761
Tít|-Borne Ty¡|us 765
Rickettsialpox 768
Louse-Borne Typhus 768
SECI|0N 21
vIkAI INF£CII0NS 0F SkIN AN0 MüC0SA 770
Poxvirus Infections 770
Molluscum Contagiosum 771
Human Oif 776
Milkei`s Nodules 778
Smallpox 779
Cowpox, Monkeypox, Tanapox 782
Cow¡ox 782
Mon|ey¡ox 783
Tana¡ox 783
Vaccinia 783
Human Papillomavirus Infections 787
Human Papillomaviius Cutaneous Infections 788
Infectious Exanthems 795
Rubella 798
Measles 800
Enteioviial Infections 803
Hand-Foot-and-Mouth Disease 803
Heipangina 804
Eiythema Infectiosum 806
Gianotti-Ciosti Syndiome 809
Dengue Fevei 810
Human Herpesviruses 813
Herpes Simplex Virus (HSV) Infection 813
Nongenital Heipes Simplex Viius Infection 818
Neonatal Heipes Simplex Viius Infection 823
Heipes Simplex Viius: Widespiead Cutaneous Infection 825
Associated with Cutaneous Immunocompiomise
Heipes Simplex Viius: Infections Associated with Systemic 827
Immunocompiomise
C0|IE|I' xvììì
Varicella Zoster Virus Infections 831
Vaiicella 833
Heipes Zostei 837
Vaiicella Zostei Viius Infections in the Immunocompiomised 846
Host
Human Herpes Virus-6 and -7 Infections: Exanthema Subitum 850
SECI|0N 28
AkIhk0F00 INS£CI 8II£S, SIINCS, AN0 CüIAN£0üS INF£CII0NS 852
Cutaneous Reactions to Arthropod Bites 852
Pediculosis 860
Pediculosis Capitis 861
Pediculosis Coipoiis 863
Pediculosis Pubis (Phthiiiasis) 865
Mite Bites and Infestations 868
Scabies 868
Cutaneous Laiva Migians 876
Water-Associated Infections and Infestations 879
Ceicaiial Deimatitis 880
Seabathei`s Eiuption 880
Envenomations Caused by Cnidaiia 882
Injuiies Caused by Echinodeims 882
SECI|0N 29
S¥SI£MIC FAkASIIIC INF£CII0NS 884
Cutaneous and Mucocutaneous Leishmaniasis 884
Trypanosomiasis 893
Ameiican Tiypanosomiasis 893
Human Afiican Tiypanosomiasis 894
Cutaneous Amebiasis and Acanthamebiasis 895
Cutaneous Amebiasis 895
Cutaneous Acanthamebiasis 895
SECI|0N 50
S£XüAII¥ IkANSMIII£0 INF£CII0NS 896
Human Papillomavirus: Mucosal Infections 900
Exteinal Genital Waits 901
HPV: Squamous Cell Caicinoma in Situ 908
and Invasive SCC of Anogenital Skin
Herpes Simplex Virus: Genital Infections 912
Syphilis 919
Piimaiy Syphilis 922
Secondaiy Syphilis 924
Latent Syphilis 928
Teitiaiy/Late Syphilis 930
Congenital Syphilis 931
HuemvphI|us ducreyI : Chancroid 931
Donovanosis 934
C0|IE|I' xìx
Ch|umydIu truchvmutIs Infections 936
Localized C. ìrat|omaìís Infection 937
Invasive C. ìrat|omaìís Infection: Lymphogianuloma veneieum 939
SECI|0N 51
hüMAN k£Ik0vIkAI INF£CII0NS AN0 MüC0CüIAN£0üS
MANIF£SIAII0NS 0F hIv[AI0S 0IS£AS£ 942
Global HIV/AIDS Pandemic 942
HIV/AIDS Disease and AIDS 942
Acute HIV/AIDS Syndrome 946
Eosinophilic Folliculitis 948
Oral Hairy Leukoplakia 950
Adverse Cutaneous Drug Eruptions in HIV/AIDS Disease 951
Abnormalities of Fat Distribution 953
Variations of Common Mucocutaneous Disorders in HIV/AIDS Disease 955
PAkI IV
SKIN SICNS CF HAIR, NAIL, AND MUCCSAL DISCRDERS
SECI|0N 52
0IS0k0£kS 0F hAIk F0IIICI£S AN0 k£IAI£0 0IS0k0£kS 961
Biology of Hair Growth Cycles 962
Classincation of Alopecia 964
Hair Loss: Alopecia 965
Paììern Haír |oss 965
À|o¡etía Àreaìa 971
Te|ogen E[[u·íum 975
Ànagen E[[u·íum 979
Cicatricial or Scarring Alopecia 981
Excess Hair Growth 989
Hiisutism 989
Hypeitiichosis 992
Infectious Folliculitis 993
SECI|0N 55
0IS0k0£kS 0F Ih£ NAII AFFAkAIüS 1000
Normal Nail Apparatus 1000
Local Disorders of Nail Apparatus 1002
Chionic Paionychia 1002
Onycholysis 1003
Gieen Nail Syndiome 1004
Onychauxis and Onychogiyphosis 1004
Psychiatric Disorders 1005
Nail Apparatus Involvement of Cutaneous Diseases 1006
Psoiiasis 1006
Lichen Planus 1008
C0|IE|I' xx
Alopecia Aieata 1008
Daiiei Disease (Daiiei-White Disease, Keiatosis 1010
Folliculaiis)
Chemical Irritant or Allergic Damage or Dermatitis 1010
Neoplasms of the Nail Apparatus 1011
Myxoid Cysts of Digits 1011
Longitudinal Melanonychia 1011
Nail Matiix Nevi 1012
Aciolentiginous Melanoma 1012
Squamous Cell Caicinoma 1012
Infections of the Nail Apparatus 1014
Bacteiial Infections 1014
Àtuìe Paronyt|ía 1014
Fe|on 1014
Fungal Infections and Onychomycosis 1014
CanJíJa Onyt|ía 1015
Tínea Unguíum/Onyt|omytosís 1016
Nail Signs of Multisystem Diseases 1021
Tiansveise oi Beau Lines 1021
Leukonychia 1021
Yellow Nail Syndiome 1022
Peiiungual Fibioma 1022
Splintei Hemoiihages 1024
Nail Fold/Peiiungual Eiythema and Telangiectasia 1024
Pteiygium Inveisum Unguium 1024
Systemic Amyloidosis 1024
Koilonychia 1026
Clubbed Nails 1026
Drug-Induced Nail Changes 1027
SECI|0N 54
0IS0k0£kS 0F Ih£ M0üIh 1028
Diseases of the Lips 1028
Angular Cheilitis (Perlèche) 1028
Conditions of the Tongue 1028
Fissuied Tongue 1028
Black oi White Haiiy Tongue 1029
Oial Haiiy Leukoplakia 1029
Migiatoiy Glossitis 1030
Diseases of the Gingiva, Periodontium, and Mucous Membranes 1030
Gingivitis and Peiiodontitis 1030
Eiosive Gingivostomatitis 1030
Lichenoid Mucositis 1030
Lichen Planus 1031
Acute Neciotizing Ulceiative Gingivitis 1032
Gingival Hypeiplasia 1032
Aphthous Ulceration 1034
Leukoplakia 1036
Erythematous Lesions and/or Leukoplakia 1037
Premalignant and Malignant Neoplasms 1038
Dysplasia and Squamous Cell Caicinoma in Situ 1038
C0|IE|I' xxì
Oial Invasive Squamous Cell Caicinoma 1038
Oial Veiiucous Caicinoma 1039
Oiophaiyngeal Melanoma 1040
Submucosal Nodules 1040
Mucocele 1040
Iiiitation Fibioma 1041
Cutaneous Odontogenic (Dental) Abscess 1042
Cutaneous Disorders Involving the Mouth 1043
Pemphigus Vulgaiis (PV) 1043
Paianeoplastic Pemphigus 1043
Bullous Pemphigoid 1043
Cicatiicial Pemphigoid 1044
Systemic Diseases Involving the Mouth 1044
Lupus Eiythematosus 1044
Behçet Disease 1044
Stevens-Johnson Syndiome / Toxic 1044
Epideimoneciolysis
Adveise Diug Reactions 1044
SECI|0N 55
0IS0k0£kS 0F Ih£ C£NIIAIIA, F£kIN£üM, AN0 ANüS 1046
Variants of Genital Anatomy 1046
Peaily Penile Papules 1046
Sebaceous Gland Piominence 1046
Angiokeiatoma 1047
Chionic Pain Syndiome 1047
Disorders Specinc to Genital Anatomy 1048
Scleiosing Lymphangitis of Penis 1048
Chionic Lymphedema of the Genitalia 1048
Plasma Cell Balanitis and Vulvitis 1048
Phimosis, Paiaphimosis, Balanitis Xeiotica Obliteians 1050
Mucocutaneous Disorders 1051
Genital(Penile/Vulvai/Anal) Lentiginoses 1051
Vitiligo and Leukodeima 1052
Psoiiasis Vulgaiis 1052
Lichen Planus 1054
Lichen Nitidus 1055
Lichen Scleiosus 1055
Migiatoiy Neciolytic Eiythema 1058
Genital Aphthous Ulceiations 1058
Eczematous Dermatitis 1059
À||ergít Conìatì Dermaìíìís 1059
Àìo¡ít Dermaìíìís, Lít|en Sím¡|ex C|ronítus, Pruríìus Àní 1060
Fixed Diug Eiuption 1061
Premalignant and Malignant Lesions 1062
Squamous Cell Caicinoma in Situ 1062
HPV-Induced Intiaepithelial Neoplasia and 1063
Squamous Cell Caicinoma in Situ
Invasive Anogenital Squamous Cell Carcinoma 1064
Invasive SCC of Penis 1064
C0|IE|I' xxìì
Invasive SCC of Vulva 1064
Invasive SCC of Cutaneous Anus 1064
Genital Veiiucous Caicinoma 1064
Malignant Melanoma of the Anogenital Region 1065
Extiamammaiy Paget Disease 1066
Kaposi Saicoma 1066
Anogenital Infections 1066
SECI|0N 56
C£N£kAIII£0 FkükIIüS WIIh0üI SkIN I£SI0NS 1068
AFF£N0IC£S 1072
APPENDIX A: °Travel" Dermatology 1072
APPENDIX B: Dermatologic Manifestations of Diseases 1072
Inßicted by Biologic Warfare/Bioterrorism
APPENDIX C: Chemical Bioterrorism and Industrial Accidents 1074
APENDIX D: Drug Use in Pregnancy 1075
Iodex 1077
°Time is change; we measure its passage by how much things alter."
NaJíne CorJímer
The Fírsì EJíìíon of this book appeaied 26 yeais ago (1983) and has been expanded paii passu with
the majoi developments that have occuiied in deimatology ovei the past two and a half decades.
Deimatology is now one of the most sought aftei medical specialties because the buiden of skin
disease has become enoimous and the many new innovative theiapies available today attiact laige
patient populations.
The Co|or Àì|as anJ Syno¡sís o[ C|íníta| Dermaìo|ogy has been used by thousands of piimaiy caie
physicians, deimatologists, inteinists, and othei health caie piovideis piincipally because it facilitates
deimatologic diagnosis by pioviding coloi photogiaphs of skin lesions and, juxtaposed, a succinct
summaiy outline of skin disoideis as well as the skin signs of systemic diseases.
The Sixth Edition has been extensively ievised, iewiitten, and expanded by the addition of new
sections. Roughly 80% of the old images have been ieplaced by new ones and additional images
have been added. Theie is a complete update of etiology, pathogenesis, management and theiapy
and theie is now an online veision. The pievious edition of the Àì|as has been tianslated into seven
languages.
Fk£FAC£
Oui secietaiy, Renate Kosma, woiked haid to meet the demands of the authois. In the piesent
McGiaw-Hill team, we appieciated the counsel of Scott Giillo, Vice Piesident and Publishei;
Anne M. Sydoi, Executive Editoi; Maiiapaz Ramos Englis, Senioi Managing Editoi; Phil Galea,
Senioi Pioduction Managei, who expeitly managed the pioduction piocess; Lindsey Zahuianec
and M. Loiiaine Andiews, the editoiial assistants, foi theii invaluble help; . Alan Bainett and
Alicia Fox, of Alan Bainett Design; and Susan Gilbeit, foi hei lovely line illustiations.
But the majoi foice behind this edition and pievious editions was Maiiapaz Ramos Englis whose
good natuie, good judgment, loyalty to the authois, and, most of all, patience, guided the authois to
make an even bettei book.
ACkN0WI£0CM£NI
INIk00üCII0N
The Co|or Àì|as anJ Syno¡sís o[ C|íníta| Der-
maìo|ogy is pioposed as a °field guide" to the
iecognition of skin disoideis and theii man-
agement. The skin is a tieasuiy of impoitant
lesions that can usually be iecognized clinically.
Gioss moiphology in the foim of skin lesions
iemains the haid coie of deimatologic diag-
nosis, and theiefoie this text is accompanied
by ovei 900 coloi photogiaphs illustiating skin
diseases, skin manifestations of inteinal dis-
eases, infections, tumois, and incidental skin
findings in otheiwise well individuals. We have
endeavoied to include infoimation ielevant
to gendei deimatology and a laige numbei of
images showing skin disease in diffeient ethnic
populations. This Àì|as coveis the entiie field of
clinical deimatology but does not include veiy
iaie syndiomes oi conditions. With iespect to
these the ieadei is iefeiied to anothei McGiaw-
Hill Publication: Fíì:¡aìrít|'s Dermaìo|ogy ín
Cenera| MeJítíne , 7th ed., 2008, edited by Klaus
Wolff, Lowell A. Goldsmith, Stephen I. Katz,
Baibaia A. Gilchiest, Amy S. Pallei, and David
J. Leffell.
This text is intended foi all physicians and
othei health caie piovideis, including medi-
cal students, deimatology iesidents, inteinists,
oncologists, and infectious disease specialists
dealing with diseases with skin manifestations.
Foi non-deimatologists, it is advisable to stait
with °Appioach to Deimatologic Diagnosis"
and °Outline of Deimatologic Diagnosis," be-
low, to familiaiize themselves with the piinci-
ples of deimatologic nomenclatuie and lines of
thought.
The Àì|as is oiganized in 4 Paits, subdi-
vided into 36 Sections, and theie aie 4 shoit
Appendices. Each section has a coloi label that
is ieflected by the bai on the top of each page.
This is to help the ieadei to find his oi hei beai-
ings iapidly when leafing thiough the book.
Also, the fiist page of each section caiiies an
°icon," i.e., a small photogiaph of a condition
that is iepiesentative foi that paiticulai section.
Each disease is labeled with little symbols to
piovide fiist-glance infoimation on incidence
(squaies) and moibidity (ciicles).
iaie low moibidity
not so common consideiable moibidity
common seiious
Foi instance, the symbols foi melanoma
aie meant to indicate that melanoma is com-
mon and seiious. Theie aie also some vaiiations
in this symbology. Foi instance, means
that the disease is iaie but may be common in
specific populations oi in endemic iegions oi in
epidemics. Anothei example indicates
that the disease causes consideiable moibid-
ity and may become seiious. In addition, each
disease is labeled with the iespective ICD9/10
codes.
Since, foi ieasons of space, not all manifesta-
tions of skin diseases and vaiiations theieof can
be shown in this piinted veision of Co|or Àì|as
anJ Syno¡sís o[ C|íníta| Dermaìo|ogy, theie is
an online veision of the book that contains
°pictuie galleiies" of most of the conditions
discussed heie. The symbol in the text iefeis
to such a pictuie galleiy in the online veision.
So, foi instance, if ieading about psoiiasis and
finding this symbol , look up the psoiiasis
pictuie galleiy in the online veision foi addi-
tional clinical images.
xxvì
Theie aie two distinct clinical situations iegaid-
ing the natuie of skin changes:
The skin changes aie íntíJenìa| findings in
we|| and í|| individuals noted duiing the iou-
tine geneial physical examination
· ° Bum¡s anJ ||emís|es ": many asympto-
matic lesions that aie medically incon-
sequential may be piesent in well and ill
peisons and aie not the ieason foi the visit
to the physician; eveiy geneial physician
should be able to iecognize these lesions to
diffeientiate them fiom asymptomatic but
impoitant, e.g., malignant, lesions.
· Im¡orìanì s|ín |esíons noì noted by the
patient but that must not be oveilooked by
the physician: e.g., atypical nevi, melanoma,
basal cell caicinoma, squamous cell caici-
noma, café-au-lait macules in von Reck-
linghausen disease, xanthomas.
The skin changes aie the t|íe[ tom¡|aínì of
the patient
· °Minoi" pioblems: e.g., localized itchy
iash, °iash," iash in gioin, nodules such as
common moles, seboiiheic keiatoses.
· °4-S": s eiious s kin s igns in s ick p atients
S£kI0üS SkIN SICNS IN SICk FAII£NIS
Cenera|ited red rash with fever
\||+| e\+u||erº
k|c|e||º|+| e\+u||erº
||uç e|up||ouº
B+c|e||+| |u|ec||ouº W||| |o\|u p|oduc||ou.
Cenera|ited red rash with b|isters and prominent
mouth |esions
E|,||er+ ru||||o|re (r+jo|)
Io\|c ep|de|r+| uec|o|,º|º
|erp||çuº
I.
II.
Bu||ouº perp||ço|d
||uç e|up||ouº
Cenera|ited red rash with pustu|es
|uº|u|+| pºo||+º|º (.ou /ur|uºc|)
||uç e|up||ouº
Cenera|ited rash with vesic|es
||ººer|u+|ed |e|peº º|rp|e\
Ceue|+||/ed |e|peº /oº|e|
\+||ce||+
||uç e|up||ouº
Cenera|ited red rash with sca|in¿ over who|e
body
E\|o||+||.e e|,|||ode|r+
Cenera|ited whea|s and soft tissue swe||in¿
u|||c+||+ +ud +uç|oeder+
Cenera|ited purpura
I||or|oc,|opeu|+
|u|pu|+ |u|r|u+uº
||uç e|up||ouº
Cenera|ited purpura that can be pa|pated
\+ºcu||||º
B+c|e||+| eudoc+|d|||º
Nu|tip|e skin infarcts
\eu|uçococcer|+
Couococcer|+
||ººer|u+|ed |u||+.+ºcu|+| co+çu|op+||,
Loca|ited skin infarcts
C+|c|p|,|+\|º
A||e|oºc|e|oº|º o||||e|+uº
A||e|oer|o||/+||ou
w+||+||u uec|oº|º
Au||p|oºp|o||p|d +u|||od, º,ud|ore
|acia| inf|ammatory edema with fever
E|,º|pe|+º
|upuº e|,||er+|oºuº
In contiast to othei fields of clinical medicine,
patients should be examined befoie a detailed
histoiy is taken because patients can see theii
lesions and thus often piesent with a histoiy
that is flawed with theii own inteipietation of
the oiigin oi causes of the skin eiuption. Also,
diagnostic accuiacy is highei when objective ex-
amination is appioached without pieconceived
ideas. Howevei, a histoiy should always be ob-
tained but if taken duiing oi aftei the visual and
physical examination, it can be stieamlined and
moie focused following the objective findings.
AFFk0ACh I0 0£kMAI0I0CIC 0IACN0SIS
INIk00üCII0N
0üIIIN£ 0F 0£kMAI0I0CIC 0IACN0SIS
xxvìì
Thus, iecognizing, analysing, and piopeily in-
teipieting skin lesions aie the sine qua non of
deimatologic diagnosis.
Fh¥SICAI £XAMINAII0N
Appearaoce Uncomfoitable, °toxic," well
vìta| Sì¿os Pulse, iespiiation, tempeiatuie
Skìo: "Iearoìo¿ to kead" The entiie skin
should be inspected and this should include
mucous membianes, genital and anal iegions,
as well as haii and nails and peiipheial lymph
nodes. Reading the skin is like ieading a text.
The basic skin lesions aie like the letteis of the
alphabet: theii shape, coloi, maigination, and
othei featuies combined will lead to woids, and
theii localization and distiibution to a sentence
oi paiagiaph. The pieiequisite of deimatologic
diagnosis is thus the iecognition of (1) the type
of skin lesion, (2) the coloi, (3) maigination, (4)
consistency, (5) shape, (6) aiiangement, and (7)
distiibution of lesions.
keco¿oìtìo¿ Ietters: Iypes oI Skìo Iesìoos
· Matu|e (Latin: matu|a, °spot") A macule
is a ciicumsciibed aiea of change in skin
coloi without elevation oi depiession. It
is thus not palpable. Macules can be well-
and ill-defined. Macules may be of any size
oi coloi (Image I-1). White, as in vitiligo;
biown, as in café-au-lait spots ; blue, as in
Mongolian spots ; oi ied, as in peimanent
vasculai abnoimalities such as poit-wine
stains oi capillaiy dilatation due to inflam-
mation (eiythema ). Piessuie of a glass slide
(Jíasto¡y ) on the boidei of a ied lesion de-
tects the extiavasation of ied blood cells. If
the iedness iemains undei piessuie fiom the
slide, the lesion is puipuiic, that is, iesults
fiom extiavasated ied blood cells; if the ied-
ness disappeais, the lesion is due to vasculai
dilatation. A iash consisting of macules is
called a matu|ar exanì|em .
· Pa¡u|e (Latin: ¡a¡u|a, °pimple") A papule
is a supeificial, elevated, solid lesion, genei-
ally consideied <0.5 cm in diametei. Most of
it is elevated above, iathei than deep within,
the plane of the suiiounding skin (Image
I-2). A papule is palpable. It may be well- oi
ill-defined. In papules the elevation is caused
by metabolic oi locally pioduced deposits , by
localized cellulai infiltiates, inflammatoiy oi
noninflammatoiy , oi by hypeiplasia of lo-
cal cellulai elements . Supeificial papules aie
shaiply defined. Deepei deimal papules have
indistinct boideis. Papules may be dome-
shaped, cone-shaped oi flat-topped (as in
lichen planus) oi consist of multiple, small,
closely packed, piojected elevations that aie
known as a ·egeìaìíon ( Image I-2 ). A iash
consisting of papules is called a ¡a¡u|ar ex-
anì|em . Papulai exanthems may be giouped
(°lichenoid") oi disseminated (dispeised).
Confluence of papules leads to the develop-
ment of laigei, usually flat-topped, ciicum-
sciibed, plateau-like elevations known as
plaques (Fiench: ¡|aque, °plate"). See below.
IMAC£ I-1 Macu|e
INIk00üCII0N
xxvììì
· P|aque A plaque is a plateau-like elevation
above the skin suiface that occupies a ielatively
laige suiface aiea in compaiison with its height
above the skin (Image I-3). It is usually well de-
fined. Fiequently it is foimed by a confluence
of papules, as in psoiiasis. Lít|ení[ítaìíon is a
less well-defined, laige plaque wheie the skin
appeais thickened and the skin maikings aie
accentuated. Lichenification occuis in atopic
deimatitis, eczematous deimatitis, psoiiasis,
lichen simplex chionicus and mycosis fun-
goides. A ¡aìt| is a baiely elevated plaque-a
lesion fitting between a macule and a plaque-
as in paiapsoiiasis oi Kaposi saicoma.
· NoJu|e (Latin: noJu|us, °small knot") A nod-
ule is a palpable, solid, iound oi ellipsoidal
lesion that is laigei than a papule (Image I-4)
and may involve the epideimis , deimis , oi sub-
cutaneous tissue. The depth of involvement and
the size diffeientiate a nodule fiom a papule.
Nodules iesult fiom inflammatoiy infiltiates ,
neoplasms , oi metabolic deposits in the deimis
oi subcutaneous tissue. Nodules may be well de-
fined (supeificial) oi ill defined (deep); if local-
ized in the subcutaneous tissue, they can often
be bettei felt than seen. Nodules can be haid oi
soft upon palpation. They may be dome-shaped
and smooth oi may have a waity suiface oi cia-
tei-like cential depiession.
· V|ea| A wheal is a iounded oi flat-topped,
pale ied papule oi plaque that is chaiacteiisti-
cally evanescent, disappeaiing within 24-48 h
(Image I-5). It is due to edema in the papillaiy
body of the deimis. Wheals may be iound,
gyiate, oi iiiegulai with pseudopods-chang-
ing iapidly in size and shape due to shifting
papillaiy edema. A iash consisting of wheals
is called an urìítaría| exanì|em oi urìítaría.
IMAC£ I-2 Fapu|e
IMAC£ I-3 F|aque
INIk00üCII0N
xxìx
· Vesít|e-Bu||a ( B|ísìer ) (Latin: ·esítu|a , °little
bladdei"; |u||a , °bubble") A vesicle (<0.5
cm) oi a bulla (>0.5 cm) is a ciicumsciibed,
elevated, supeificial cavity containing fluid
(Image I-6). Vesicles aie dome-shaped (as in
contact deimatitis, deimatitis heipetifoimis),
umbilicated (as in heipes simplex), oi flaccid
(as in pemphigus). Often the ioof of a vesi-
cle/bulla is so thin that it is tianspaient, and
the seium oi blood in the cavity can be seen.
Vesicles containing seium aie yellowish; those
containing blood fiom ied to black. Vesicles
and bullae aiise fiom a cleavage at vaiious
levels of the supeificial skin; the cleavage may
be subcoineal oi within the visible epideimis
(i.e., intiaepideimal vesication) oi at the
epideimal-deimal inteiface (i.e., subepidei-
mal), as in Image I-6. Since vesicles/bullae
aie always supeificial they aie always well de-
fined. A iash consisting of vesicles is called a
·esítu|ar exanì|em ; a iash consisting of bullae
a |u||ous exanì|em.
IMAC£ I-4 Nodu|e
IMAC£ I-5 Whea|
IMAC£ I-6 vesìc|e
INIk00üCII0N
xxx
· Pusìu|e (Latin: ¡usìu|a , °pustule") A pus-
tule is a ciicumsciibed, supeificial cavity of
the skin that contains a puiulent exudate
(Image I-7), which may be white, yellow,
gieenish-yellow, oi hemoiihagic. Pustules
thus diffei fiom vesicles in that they aie not
cleai but have a tuibid content. This piocess
may aiise in a haii follicle oi independently.
Pustules may vaiy in size and shape. Pus-
tules aie usually dome-shaped, but follicu-
lai pustules aie conical and usually contain
a haii in the centei. The vesiculai lesions
of heipes simplex and vaiicella zostei vi-
ius infections may become pustulai. A iash
consisting of pustules is called a ¡usìu|ar
exanì|em.
· Crusìs (Latin: trusìa , °iind, baik, shell")
Ciusts develop when seium, blood, oi puiu-
lent exudate diies on the skin suiface (Image
I-8). Ciusts may be thin, delicate, and fiiable
(À ) oi thick and adheient . Ciusts aie yellow
when foimed fiom diied seium; gieen oi
yellow-gieen when foimed fiom puiulent
exudate; oi biown, daik ied, oi black when
foimed fiom blood. Supeificial ciusts occui
as honey-coloied, delicate, glistening paiticu-
lates on the suiface and aie typically found
in impetigo. When the exudate involves the
entiie epideimis, the ciusts may be thick and
adheient , and if it is accompanied by neciosis
of the deepei tissues (e.g., the deimis), the
condition is known as etì|yma .
IMAC£ I-T Fustu|e
IMAC£ I-8 Crust
· Sta|es (squames) (Latin: squama , °scale")
Scales aie flakes of stiatum coineum (Image
I-9). They may be laige (like membianes ,
tiny (like dust), pityiiasifoim (Gieek: ¡íìyron ,
°bian"), adheient, oi loose. A iash consisting
of papules with scales is called a ¡a¡u|osqua-
mous exanì|em.
INIk00üCII0N
xxxì
· Erosíon An eiosion is a defect only of the epi-
deimis, not involving the deimis (Image I-10);
in contiast to an ulcei, which always heals with
scai foimation (see below), an eiosion heals
without a scai. An eiosion is shaiply defined
and is ied and oozes. Theie aie supeificial
eiosions, which aie subcoineal oi iun thiough
the epideimis, and deep eiosions, the base of
which is the papillaiy body (Image I-10). Ex-
cept foi physical abiasions, eiosions aie always
the iesult of intiaepideimal oi subepideimal
cleavage and thus of vesicles oi bullae.
· U|ter (Latin: u|tus , °soie") An ulcei is a skin
defect that extends into the deimis oi deepei
(Image I-11) into the subcutis and always oc-
cuis within pathologically alteied tissue. An
ulcei is theiefoie always a secondaiy phenom-
enon. The pathologically alteied tissue giving
iise to an ulcei is usually seen at the boidei
oi the base of the ulcei and is helpful in de-
teimining its cause. Othei featuies helpful in
this iespect aie whethei boideis aie elevated,
undeimined, haid, oi soggy; location of the ul-
cei; dischaige; and any associated topogiaphic
featuies, such as nodules, exoiiations, vaiicosi-
ties, haii distiibution, piesence oi absence of
sweating, and aiteiial pulses. Ulceis always
heal with scai foimation.
· Star A scai is the fibious tissue ieplacement
of the tissue defect by pievious ulcei oi a
wound. Scais can be hypeitiophic and haid
(Image I-12 ) oi atiophic and soft with a thin-
ning oi loss of all tissue compaitments of the
skin (Image I-12 ).
· Àìro¡|y This iefeis to a diminution of
some oi all layeis of the skin (Image I-
13). Epideimal atiophy is manifested by a
thinning of the epideimis, which becomes
tianspaient, ievealing the papillaiy and sub-
papillaiy vessels ; theie aie loss of skin tex-
tuie and cigaiette papei-like wiinkling. In
deimal atiophy theie aie loss of connective
tissue of the deimis and depiession of the
lesion ( Image I-13 ).
IMAC£ I-9 Sca|e
IMAC£ I-10 £rosìoo
INIk00üCII0N
xxxìì
· Cysì A cyst is a cavity containing liquid
oi solid oi semisolid (Image I-14) mateiials
and may be supeificial oi deep. Visually it
appeais like a spheiical, most often dome-
shaped papule oi nodule, but upon palpation
it is iesilient. It is lined by an epithelium and
often has a fibious capsule; depending on its
contents it may be skin coloied, yellow, ied,
oi blue. An epideimal cyst pioducing keiati-
naceous mateiial and a pilai cyst that is lined
by a multilayeied epithelium aie shown in
Image I-14.
IMAC£ I-11 ü|cer
IMAC£ I-12 Scar
IMAC£ I-13 Atrophy
INIk00üCII0N
xxxììì
Shapìo¿ Ietters ìoto Words: Further
Characterìtatìoo oI IdeotìIìed Iesìoos
· Co|or Pink, ied, puiple ¦puipuiic lesions
do not blanch with piessuie with a glass slide
(diascopy)], white, tan, biown, black, blue,
giey, yellow. The coloi can be unifoim oi
vaiiegated.
· Margínaìíon Well defined (can be tiaced
with the tip of a pencil), ill defined.
· S|a¡e Round, oval, polygonal, polycy-
clic, annulai (iing-shaped), iiis, seipiginous
(snakelike), umbilicated.
· Pa|¡aìíon Considei (1) tonsísìenty (soft, fiim,
haid, fluctuant, boaidlike); (2) Je·íaìíon ín
ìem¡eraìure (hot, cold); and (3) mo|í|íìy . Note
piesence of ìenJerness , and estimate the Je¡ì|
of the lesion (i.e., deimal oi subcutaneous).
Formìo¿ Seoteoces aod üoderstaodìo¿ the Iext:
£va|uatìoo oI Arrao¿emeot, Fatteros, aod
0ìstrìbutìoo
· Num|er Single oi multiple lesions.
· Àrrangemenì Multiple lesions may be (1)
grou¡eJ : heipetifoim, aicifoim, annulai, ie-
ticulated (net-shaped), lineai, seipiginous
(snakelike); oi (2) JíssemínaìeJ : scatteied
disciete lesions.
· Con[|uente Yes oi no.
· Dísìrí|uìíon Considei (1) exìenì : iso-
lated (single lesions), localized, iegional,
geneialized, univeisal, and (2) ¡aììern : sym-
metiic, exposed aieas, sites of piessuie, intei-
tiiginous aiea, folliculai localization, iandom,
following deimatomes oi Blaschko lines.
Table I-1 piovides an algoiithm showing how
to pioceed.
hISI0k¥
0emo¿raphìcs Age, iace, sex, occupation.
hìstory
1. Constitutional symptoms
· °Acute illness" syndiome: headaches, chills,
feveiishness, weakness
· °Chionic illness" syndiome: fatigue, weak-
ness, anoiexia, weight loss, malaise
2. History of skin lesions. Seven key ques-
tions:
· When: Onset
· Wheie: Site of onset
· Does it itch oi huit: Symptoms
· How has it spiead (pattein of spiead):
Evolution
· How have individual lesions changed:
Evolution
· Piovocative factois: Heat, cold, sun, exei-
cise, tiavel histoiy, diug ingestion, pieg-
nancy, season
· Pievious tieatment(s): Topical and sys-
temic,
3. General history of present illness as indi-
cated by clinical situation, with particular
attention to constitutional and prodromal
symptoms
4. Past medical history
· Opeiations
· Illnesses (hospitalized:)
· Alleigies, especially diug alleigies
· Medications (piesent and past)
· Habits (smoking, alcohol intake, diug
abuse)
· Atopic histoiy (asthma, hay fevei, ec-
zema)
IMAC£ I-14 Cyst
INIk00üCII0N
xxxìv
|deot|Iy |es|oos
|s |es|oo so||tary or are there m0|t|p|e |es|oos?
Nac0|e
º portwiue staiu*
º er]thema migraus
Pap0|elood0|e
º dermal uevus
º oasal cell
carciuoma
º uodular
melauoma
P|ag0e
º licheu simplex
chrouicus
º Boweu disease
spreadiug
melauoma
0|cer
º oasal cell
carciuoma
º diaoetic ulcer
º primar] chaucre
of s]philis
Nac0|ar
º solar
leutigiues
eruptiou
Nac0|ar
º viral exauthem
º drug eruptiou
P|ag0e
º psoriasis
º m]cosis
fuugoides
hod0|ar
º metastatic
caucer
P0st0|ar
º folliculitis
oaroae
º herpes zoster
º impetigo
*Bulleted couditious are examples
Pap0|ar
º coud]lomata
accumiuata
º s]riugomas
º licheu plauus
Ves|c0|arl
b0||o0s
º herpes
zoster
º herpes
simplex
Pap0|ar
º psoriasis
º licheu plauus
º secoudar] s]philis
Ves|c0|arlb0||o0s
º varicella
º oullous
pemphigoid
P0st0|ar
º pustular
psoriasis
º smallpox
hod0|ar
º metastatic
melauoma
º lipomas
Loca||zed
N0|t|p|e
6eoera||zed
So||tary
IA8I£ I-1 A|¿orithm for Eva|uatin¿ Skin Lesions
5. Family medical history (particularly of
psoriasis, atopy, melanoma, xanthomas, tu-
berous sclerosis)
6. Social history, with particular reference
to occupation, hobbies, exposures, travel,
injecting drug use
7. Sexual history: history of risk factors of
HIV: blood transfusions, IV drugs, sexually
active, multiple partners, sexually trans-
mitted disease?
k£vI£W 0F S¥MFI0MS
This should be done as indicated by the clinical
situation, with paiticulai attention to possible
connections between signs and disease of othei
oigan systems (e.g., iheumatic complaints, my-
algias, aithialgias, Raynaud phenomenon, sicca
symptoms).
INIk00üCII0N
xxxv
SF£CIAI I£ChNIÇü£S üS£0 IN CIINICAI
£XAMINAII0N
Magní[ítaìíon wíì| |anJ |ens. To examine le-
sions foi fine moiphologic detail, it is necessaiy
to use a magnifying glass (hand lens) (7×) oi
a binoculai micioscope (5 to 40). Magni-
fication is especially helpful in the diagnosis
of lupus eiythematosus (folliculai plugging),
lichen planus (Wickham stiiae), basal cell cai-
cinomas (tianslucence and telangiectasia), and
melanoma (subtle changes in coloi, especially
giay oi blue); this is best visualized aftei ap-
plication of a diop of mineial oil. Use of the
deimatoscope is discussed below (see °Dei-
moscopy").
O||íque |íg|ìíng of the skin lesion, done in
a daikened ioom, is often iequiied to detect
slight degiees of elevation oi depiession, and
it is useful in the visualization of the suiface
configuiation of lesions and in estimating the
extent of the eiuption.
Su|JueJ |íg|ìíng in the examining ioom
enhances the contiast between ciicumsciibed
hypopigmented oi hypeipigmented lesions and
noimal skin.
VooJ |am¡ (ultiaviolet long-wave light,
°black" light) is valuable in the diagnosis of
ceitain skin and haii diseases and of poiphyiia.
With the Wood lamp (365 nm), fluoiescent pig-
ments and subtle coloi diffeiences of melanin
pigmentation can be visualized; the Wood lamp
also helps to estimate vaiiation in the lightness
of lesions in ielation to the noimal skin coloi
in both daik-skinned and faii-skinned peisons;
e.g., the lesions seen in tubeious scleiosis and
tinea veisicoloi aie hypomelanotic and aie not
as white as the lesions seen in vitiligo, which
aie amelanotic. Ciicumsciibed hypeimelanosis,
such as a fieckle and melasma, is much moie
evident (daikei) undei the Wood lamp. By con-
tiast, deimal melanin, as in a Mongolian sacial
spot, does not become accentuated undei the
Wood lamp. Theiefoie, it is possible to localize
the site of melanin by use of the Wood lamp;
|owe·er, ì|ís ís more Jí[[ítu|ì or noì ¡ossí||e ín
¡aìíenìs wíì| |rown or ||at| s|ín.
Wood lamp is paiticulaily useful in the
detection of the fluoiescence of deimatophy-
tosis in the haii shaft (gieen to yellow) and of
eiythiasma (coial ied). A piesumptive diagno-
sis of poiphyiia can be made if a pinkish-ied
SF£CIAI CIINICAI AN0 IA80kAI0k¥ AI0S I0 0£kMAI0I0CIC
0IACN0SIS
fluoiescence is demonstiated in uiine examined
with the Wood lamp; addition of dilute hydio-
chloiic acid intensifies the fluoiescence.
Díasto¡y consists of fiimly piessing a micio-
scopic slide oi a glass spatula ovei a skin lesion.
The examinei will find this pioceduie of special
value in deteimining whethei the ied coloi of
a macule oi papule is due to capillaiy dilata-
tion (eiythema) oi to extiavasation of blood
(puipuia) that does not blanch. Diascopy is also
useful foi the detection of the glassy yellow-
biown appeaiance of papules in saicoidosis,
tubeiculosis of the skin, lymphoma, and gianu-
loma annulaie.
Dermosto¡y (also called e¡í|umínestente mí-
trosto¡y ). A hand lens with built-in lighting and
a magnification of 10× to 30× is called a Jerma-
ìosto¡e and peimits the noninvasive inspection
of deepei layeis of the epideimis and beyond.
This is paiticulaily useful in the distinction
of benign and malignant giowth patteins in
pigmented lesions. Dígíìa| Jermosto¡y is pai-
ticulaily useful in the monitoiing of pigmented
skin lesions because images aie stoied electioni-
cally and can be ietiieved and examined at a
latei date to peimit compaiison quantitatively
and qualitatively and to detect changes ovei
time. Digital deimoscopy uses computei image
analysis piogiams that piovide (1) objective
measuiements of changes; (2) iapid stoiage,
ietiieval, and tiansmission of images to ex-
peits foi fuithei discussion (teledeimatology);
and (3) extiaction of moiphologic featuies foi
numeiical analysis. Deimoscopy and digital
deimoscopy iequiie special tiaining.
CIINICAI SICNS
Daríer sígn is °positive" when a biown maculai
oi a slightly papulai lesion of uiticaiia pigmen-
tosa (mastocytosis) becomes a palpable wheal
aftei being vigoiously iubbed with an instiu-
ment such as the blunt end of a pen. The wheal
may not appeai foi 5-10 min.
Àus¡íì: sígn is °positive" when slight sciatch-
ing oi cuietting of a scaly lesion ieveals punc-
tate bleeding points within the lesion. This
suggests psoiiasis, but it is not specific.
The Ní|o|s|y ¡|enomenon is positive when
the epideimis is dislodged fiom the deimis by
lateial, sheaiing piessuie with a fingei, iesulting
INIk00üCII0N
xxxvì
in an eiosion. It is an impoitant diagnostic sign
in acantholytic disoideis such as pemphigus oi
the staphylococcal scalded skin (SSS) syndiome
oi othei blisteiing oi epideimoneciotic disoi-
deis, such as toxic epideimal neciolysis.
CIINICAI I£SIS
Paìt| ìesìíng is used to document and validate
a diagnosis of alleigic contact sensitization and
identify the causative agent. Substances to be
tested aie applied to the skin in shallow cups
(Finn chambeis), affixed with a tape and left
in place foi 24-48 h. Contact hypeisensitivity
will show as a papulai vesiculai ieaction that
develops within 48-72 h when the test is iead.
It is a unique means of in vivo iepioduction of
disease in diminutive piopoitions, foi sensitiza-
tion affects all the skin and may theiefoie be
elicited at any cutaneous site. The patch test is
easiei and safei than a °use test" with a ques-
tionable alleigen, foi test items can be applied
in low concentiations in small aieas of skin foi
shoit peiiods of time (see Section 2).
P|oìo¡aìt| ìesìíng is a combination of patch
testing and UV iiiadiation of the test site and
is used to document photoalleigy (see Section
10).
Prít| ìesìíng is used to deteimine type I allei-
gies. A diop of a solution containing a minute
concentiation of the alleigen is placed on the
skin and the skin is pieiced thiough this diop
with a needle. Pieicing should not go beyond
the papillaiy body. A positive ieaction will ap-
peai as a wheal within 20 min. The patient has
to be undei obseivation foi possible anaphy-
laxis.
Àteìow|íìeníng facilitates detection of sub-
clinical penile oi vulvai waits. Gauze satuiated
with 5% acetic acid (white vinegai) is wiapped
aiound the glans penis oi used on the ceivix
and anus. Aftei 5-10 min, the penis oi vulva is
inspected with a 10 hand lens. Waits appeai as
small white papules.
IA80kAI0k¥ I£SIS
Mìcroscopìc £xamìoatìoo oI Sca|es, Crusts,
Serum, aod haìr
Cram sìaíns of smeais and tu|ìures o[ exuJaìes
anJ o[ ìíssue míntes should be made in lesions
suspected of being bacteiial oi yeast ( CanJíJa
a||ítans ) infections. Ulceis and nodules iequiie
a scalpel biopsy in which a wedge of tissue
consisting of all thiee layeis of skin is obtained;
the biopsy specimen is divided into one-half foi
histopathology and one-half foi cultuie. This is
minced in a steiile moitai and then cultuied foi
bacteiia (including typical and atypical myco-
bacteiia) and fungi.
Mítrosto¡ít examínaìíon foi mycelia should
be made of the ioofs of vesicles oi of scales (the
advancing boideis aie piefeiable) oi of the haii
in deimatophytoses. The tissue is cleaied with
10-30% KOH and waimed gently. Hyphae and
spoies will light up by theii biiefiingence (Fig.
25-1). Fungal cultuies with Sabouiaud medium
should be made (see Section 25).
Mítrosto¡ít examínaìíon o[ te||s o|ìaíneJ [rom
ì|e |ase o[ ·esít|es (Tzanck piepaiation) may
ieveal the piesence of acantholytic cells in the
acantholytic diseases (e.g., pemphigus oi SSS
syndiome) oi of giant epithelial cells and multi-
nucleated giant cells (containing 10-12 nuclei)
in heipes simplex, heipes zostei, and vaiicella.
Mateiial fiom the base of a vesicle obtained by
genì|e cuiettage with a scalpel is smeaied on a
glass slide, stained with eithei Giemsa oi Wiight
stain oi methylene blue, and examined to de-
teimine whethei theie aie acantholytic oi giant
epithelial cells, which aie diagnostic (Fig. 27-
27). In addition, cultuie, immunofluoiescence
tests, oi polymeiase chain ieaction foi heipes
have to be oideied.
La|oraìory Jíagnosís o[ sta|íes . The diagnosis
is established by identification of the mite, oi
ova oi feces, in skin sciapings iemoved fiom
the papules oi buiiows (see Section 28). Using
a steiile scalpel blade on which a diop of steiile
mineial oil has been placed, apply oil to the sui-
face of the buiiow oi papule. Sciape the papule
oi buiiow vigoiously to iemove the entiie top
of the papule; tiny flecks of blood will appeai
in the oil. Tiansfei the oil to a micioscopic slide
and examine foi mites, ova, and feces. The mites
aie 0.2-0.4 mm in size and have foui paiis of
legs (see Section 28).
8ìopsy oI the Skìo
Biopsy of the skin is one of the simplest, most
iewaiding diagnostic techniques because of the
easy accessibility of the skin and the vaiiety
of techniques foi study of the excised speci-
men (e.g., histopathology, immunopathology,
polymeiase chain ieaction, election micios-
copy).
Selection of the site of the biopsy is based pii-
maiily on the stage of the eiuption, and eaily le-
sions aie usually moie typical; this is especially
impoitant in vesiculobullous eiuptions (e.g.,
pemphigus, heipes simplex), in which the le-
sion should be no moie than 24 h old. Howevei,
INIk00üCII0N
xxxvìì
oldei lesions (2-6 weeks) aie often moie chai-
acteiistic in discoid lupus eiythematosus.
A common technique foi diagnostic biopsy is
the use of a 3- to 4-mm punch, a small tubulai
knife much like a coiksciew, which by iotat-
ing movements between the thumb and index
fingei cuts thiough the epideimis, deimis, and
subcutaneous tissue; the base is cut off with
scissois. If immunofluoiescence is indicated
(e.g., as in bullous diseases oi lupus eiythema-
tosus), a special medium foi tianspoit to the
laboiatoiy is iequiied.
Foi nodules, howevei, a laige wedge should
be iemoved by excision including subcutaneous
tissue. Fuitheimoie, when indicated, lesions
should be bisected, one-half foi histology and
the othei half sent in a steiile containei foi
bacteiial and fungal cultuies oi in special fixa-
tives oi cell cultuie media, oi fiozen foi immu-
nopathologic examination.
Specimens foi light micioscopy should be
fixed immediately in buffeied neutial foimalin.
A biief but detailed summaiy of the clinical
histoiy and desciiption of the lesions should
accompany the specimen. Biopsy is indicated
in a|| skin lesions that aie suspected of being
neoplasms, in all bullous disoideis with immun-
ofluoiesence used simultaneously, and in all dei-
matologic disoideis in which a specific diagnosis
is not possible by clinical examination alone.
INIk00üCII0N
This page intentionally left blank
| A k I |
DI5OkDEk5 PkE5ENIINC
IN IHE 5KIN AND
MUCOU5 MEMßkANE5
DI5OkDEk5 PkE5ENIINC
IN IHE 5KIN AND
MUCOU5 MEMßkANE5
2
0IS0k0£kS 0F S£8AC£0üS
AN0 AF0CkIN£ CIAN0S
S E C I | 0 N |
2
Au |u||+rr+||ou o| p||oºe|+ceouº uu||º, .e|, cor·
rou
Appe+|º |u ce||+|u |od, +|e+º (|+ce, ||uu|, |+|e|,
|u||oc|º)
\oº| ||equeu||, |u +do|eºceu|º
\+u||eº|º +º coredoueº, p+pu|opuº|u|eº, uod·
u|eº, +ud c,º|º
keºu||º |u p|||ed, dep|eººed, o| |,pe|||op||c
ºc+|º
|C|·9. 10o.¹

|C|·¹0. |10.0
ACN£ vüICAkIS (C0MM0N ACN£) AN0 C¥SIIC ACN£
£FI0£MI0I0C¥
0ccurreoce Veiy common, affecting appioxi-
mately 85% of young people.
A¿e oI 0oset Pubeity-10 to 17 yeais in fe-
males, 14 to 19 in males; howevei, may appeai
fiist at 25 yeais oi oldei.
Sex Moie seveie in males than in females.
kace Lowei incidence in Asians and Afiicans.
Ceoetìc Aspects Multifactoiial genetic back-
giound. Familial piedisposition: majoiity of
individuals with cystic acne have paient(s) with
a histoiy of seveie acne. Seveie acne may be
associated with XYY syndiome.
FAIh0C£N£SIS
Key [atìors aie folliculai keiatinization, andio-
gens, and Pro¡íoní|atìeríum atnes (Image 1-1).
Acne iesults fiom a change in the keiat-
inization pattein in the pilosebaceous unit,
with the keiatinous mateiial becoming moie
dense and blocking secietion of sebum. These
keiatin plugs aie called tomeJones and iep-
iesent the °time bombs" of acne. Linoleic acid,
which iegulates keiatinocyte piolifeiation, is
decieased in acne. Comedonal plugging and a
complex inteiaction between andiogens and
bacteiia ( P. atnes ) in the plugged piloseba-
ceous units lead to inflammation. Andiogens
(qualitatively and quantitatively noimal in the
seium) stimulate sebaceous glands to pioduce
laigei amounts of sebum. Bacteiia contain
lipase, which conveits lipid into fatty acids, and
pioduce pioinflammatoiy mediatois, ¦intei-
leukin 1, tumoi neciosis factoi TNF ]. Fatty
acids and pioinflammatoiy mediatois cause a
steiile inflammatoiy iesponse to the piloseba-
ceous unit. The distended follicle walls bieak,
and the contents (sebum, lipids, fatty acids,
keiatin, bacteiia) entei the deimis, piovoking
an inflammatoiy and foieign-body iesponse
(papule, pustule, nodule). Ruptuie plus intense
inflammation lead to scais.
Cootrìbutory Factors Acnegenic mineial oils,
iaiely dioxin and otheis.
Drugs Lithium, hydantoin, isoniazid, glucocoi-
ticoids, oial contiaceptives, iodides, biomides
and andiogens (e.g., testosteione), danazol.
Others Emoìíona| sìress can definitely cause
exaceibations. Ott|usíon and ¡ressure on the
skin, such as by leaning face on hands, ·ery
ím¡orìanì and often uniecognized exaceibating
factoi ( atne met|aníta ). Acne is not caused by
chocolate oi fatty foods oi, in fact, by any kind
of food.
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos Weeks to months.
Seasoo Often woise in fall and wintei.
Symptoms Pain in lesions (especially nodulo-
cystic type).
Skìo Iesìoos ComeJones -open (blackheads)
oi closed (whiteheads); tomeJona| atne
(Fig. 1-1). Pa¡u|es and ¡a¡u|o¡usìu|es -i.e.,
a papule topped by a pustule; ¡a¡u|o¡usìu|ar
atne (Fig. 1-2). NoJu|es oi tysìs -1-4 cm in
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 3
FICük£ 1-1 Acoe vu|¿arìs: comedooes Coredoueº +|e |e|+||u p|uçº ||+| |o|r W||||u |o|||cu|+| oº||+,
||equeu||, +ººoc|+|ed W||| ºu||ouud|uç e|,||er+ +ud puº|u|e |o|r+||ou. Coredoueº +ººoc|+|ed W||| ºr+|| oº||+
+|e |e|e||ed |o +º c|oºed coredoueº o| 'W|||e |e+dº¨, ||oºe +ººoc|+|ed W||| |+|çe oº||+ +|e |e|e||ed |o +º opeu
coredoueº o| '||+c| |e+dº.¨ Coredoueº +|e |eº| ||e+|ed W||| |op|c+| |e||uo|dº.
FICük£ 1-2 20-year-o|d ma|e |u |||º c+ºe o| p+pu|opuº|u|+| +cue, ºore |u||+rr+|o|, p+pu|eº |ecore
uodu|+| +ud ||uº |ep|eºeu| e+||, º|+çeº o| uodu|oc,º||c +cue.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 4
diametei (Fig. 1-3); noJu|otysìít atne . Soft
nodules iesult fiom iepeated folliculai iuptuies
and ieencapsulations with inflammation,
abscess foimation, and foieign-body ieaction.
Cysts aie actually pseudocysts as they aie not
lined by epithelium but iepiesent fluctuating
abscesses (Image 1-1). Round isolated single
nodules and cysts coalesce to lineai mounds
and sinus tiacts (Fig. 1-4). Sínuses : diaining
epithelial-lined tiacts, usually with nodulai
acne. Stars : atiophic depiessed (often pitted) oi
hypeitiophic (at times, keloidal). Se|orr|ea of
the face and scalp often piesent and sometimes
seveie. Foi moie clinical pictuies, see acne
pictuie galleiy on online veision.
SItes v] PredI|ectIvn Face, neck, tiunk, uppei
aims, buttocks.
Specìa| Forms
Acoe Coo¿|obata Seveie cystic acne (Figs.
1-4 and 1-5) with moie involvement of the
tiunk than the face. Coalescing nodules, cysts,
abscesses, and ulceiation; occuis also on but-
tocks. Spontaneous iemission is long delayed.
Raiely, acne conglobata seen in XYY genotype
(tall males, slightly mentally ietaided, with
IMAC£ 1-1 A-0. Acoe patho¿eoesìs ¦||or /+euç|e|u A| e| +|. Acue .u|ç+||º +ud +cue||o|r e|up||ouº, |u
wo||| K e| +| (edº). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, 1|| ed. |eW \o||, \cC|+W·n|||, 2003.|
aggiessive behavioi) oi in the polycystic ovaiy
syndiome.
Acoe Fu|mìoaos Teenage boys (ages 13 to 17).
Àtuìe onseì , seveie cystic acne with concomitant
suppuiation and always u|teraìíon ; also piesent
aie malaise, fatigue, fevei, geneialized aithial-
gias, leukocytosis, and elevated eiythiocyte
sedimentation iate.
SAFh0 Syodrome Synovitis, acne, acne ful-
minans, ¡almoplantai pustulosis, |idiadenitis
suppuiativa, hypeiostosis, and osteitis. Raie.
FAFA Syodrome Steiile ¡yogenic aithiitis,
¡yodeima gangienosum acne. An inheiited au-
toinflammatoiy disoidei; veiy iaie.
Iropìca| Acoe Flaie of acne, usually with se-
veie folliculitis, inflammatoiy nodules, and
diaining cysts on tiunk and buttocks in tiopical
climates; secondaiy infection with Sìa¡|y|otot-
tus aureus .
Acoe wìth Facìa| £dema Associated with iecal-
citiant, disfiguiing midline facial edema. Woody
induiation with and without eiythema.
Acoe ìo the Adu|t Womao Peisistent acne in
an (often) hiisute female with oi without ír-
regu|ar menses needs an evaluation foi hypei-
secietion of adienal and ovaiian andiogens:
N|crocomadona
º hyparkaratot|c
|nfund|bu|um
º cohas|va cornaocytas
º sabum sacrat|on
0omadona
º accumu|at|on of
shad cornaocytas
and sabum
º d||at|on of fo|||cu|ar
ost|um
|nßammatory papu|a/
pustu|a
º furthar axpans|on
of fo|||cu|ar un|t
º pro||farat|on of
Prcpicnibacterium acnes
par|fo|||cu|ar |nßammat|on
hodu|a
º ruptura of fo|||cu|ar wa||
º markad par|fo|||cu|ar
|nßammat|on
º scarr|ng
A 8 0 0
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 5
total testosteione, fiee testosteione, and/oi de-
hydioepiandiosteione sulfate (DHEAS) (e.g., in
the polycystic ovaiy syndiome).
keca|cìtraot Acoe Can be ielated to congenital
adienal hypeiplasia (11 - oi 21 - hydioxylase
deficiencies).
Acoe £xcorìée Mild acne, usually in young
women, associated with extensive excoiiations
and scaiiing due to emotional and psychological
pioblems (obsessive compulsive disoidei).
Neooata| Acoe On nose and cheeks in new-
boins oi infants, ielated to glandulai develop-
ment; tiansient.
0ccupatìooa| Acoe Due to exposuie to tai de-
iivatives, cutting oils, chloiinated hydiocaibons
(see °Chloiacne," below). Laige comedones,
inflammatoiy papules and cysts; not iestiicted
to piedilection sites of acne but can appeai on
othei (coveied) body sites.
Ch|oracoe Due to exposuie to chloiinated
aiomatic hydiocaibons in electiical conductois,
insecticides, and heibicides. Sometimes veiy
seveie due to industiial accidents oi intended
poisoning (e.g., dioxin).
Acoe Cosmetìca Due to comedogenic cos-
metics.
FICük£ 1-3 Nodu|ocystìc acoe A º,rre|||c d|º||||u||ou |u ||e |+ce o| + |eeu+çe |o,. I||º |r+çe c|e+||,
º|oWº ||+| e.eu uodu|oc,º||c +cue º|+||º W||| coredoueº-|o|| opeu +ud c|oºed coredoueº c+u |e ºeeu |u |||º
|+ce-||+| ||eu ||+uº|o|r |u|o p+pu|opuº|u|+| |eº|ouº, W||c| eu|+|çe +ud co+|eºce e.eu|u+||, |o |e+d |o uodu|oc,º||c
+cue. || |º uo| ºu|p||º|uç ||+| ||eºe |eº|ouº +|e .e|, p+|u|u|, +ud || |º uude|º|+ud+||e ||+| uodu|oc,º||c +cue +|ºo
ºe.e|e|, |rp+c|º ||e ºoc|+| |||e o| ||eºe +do|eºceu|º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 6
decade oi oldei. Flaies occui in the wintei
and with the onset of menses. The sequela is
scaiiing (foi clinical examples see , which
should be avoided by piopei tieatment, es¡e-
tía||y wíì| ora| ísoìreìínoín ear|y ín ì|e tourse o[
ì|e Jísease (see below).
MANAC£M£NI
The psychological impact of acne (peiceived
cosmetic disfiguiement) should be assessed
individually in each patient and theiapy modi-
fied accoidingly. The goal of theiapy is to
iemove the plugging of the pilai diainage,
ieduce sebum pioduction, and tieat bacteiial
colonization.
Mì|d Acoe
Topical antibiotics (clindamycin and eiyth-
iomycin)
Benzoyl peioxide gels (2%, 5%, oi 10%)
Topical ietinoids (tietinoin, adapalene) ie-
quiie detailed instiuctions iegaiding giad-
ual incieases in concentiation fiom 0.01%
to 0.025% to 0.05% cieam/gel oi liquid.
Aftei impiovement, medication is ieduced
to the lowest effective maintenance.
Impiovement occuis ovei a peiiod of months
(2-5) but may take even longei foi noninflamed
comedones. Topical ietinoids aie applied in the
evening; topical antibiotics and benzoyl pei-
oxide gels aie applied duiing the day.
Combination theiapy is best, using benzoyl
peioxide-eiythiomycin gels ¡|us topical ietin-
oids (tietinoin or tazaiotene gel, adapalene).
Noìe. acne suigeiy (extiactions of comedones)
is helpful only when piopeily done and aftei
pietieatment with topical ietinoids.
Moderate Acoe Oial antibiotics aie added to the
above iegimen. Most effective antibiotic is minoc-
ycline, 50 -100 mg twice daily, oi doxycycline, 50 -
100 mg twice daily, and this is tapeied to 50- mg/d
as acne lessens. In females, modeiate acne can be
contiolled with high doses of oial estiogens com-
bined with piogesteione oi antiandiogens, but ie-
cuiiences aie the iule aftei cessation of tieatment.
Ceiebiovasculai accidents aie a seiious iisk.
Noìe. foi inflammatoiy cysts and nodules, in-
tialesional tiiamcinolone (0.05 mL of a 3- to 5-
mg/mL suspension) is indicated.
Severe Acoe In addition to the topical tieat-
ment outlined above, systemic tieatment with
isotietinoin is indicated foi cystic oi conglo-
bate acne oi foi acne iefiactoiy to tieatment.
This ietinoid inhibits sebaceous gland func-
tion and keiatinization and is veiy effective.
Fomade Acoe On the foiehead, usually in Af-
iicans applying pomade to haii.
Acoe Mechaoìca Flaies of pieexisting acne in
face, because of leaning face on hands, oi on
foiehead, fiom piessuie of football helmet.
Acoe-Iìke Coodìtìoos
Steroìd Acoe Following systemic oi topical
glucocoiticoids. Monomoiphous folliculitis-
small eiythematous papules and pustules wíì|-
ouì comedones.
0ru¿-Ioduced Acoe Monomoiphous acne-
like eiuption due to phenytoin, lithium, isoni-
azid, high-dose vitamin B complex, epideimal
giowth factoi inhibitois (see Section 22), halo-
genated compounds. No comedones.
Acoe Aestìva|ìs Papulai eiuption aftei sun ex-
posuie (°Malloica acne"). Usually on foiehead,
shouldeis, aims, neck, and chest. No come-
dones. Pathogenesis unknown.
Cram-Ne¿atìve Fo||ìcu|ìtìs Multiple tiny yellow
pustules develop on top of acne vulgaiis as a ie-
sult of long-teim antibiotic administiation.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Noìe : Comedones aie iequiied foi diagnosis of
any type of acne. Comedones aie not a featuie
of acne-like conditions (above) and of the con-
ditions listed below.
Face S. aureus folliculitis, pseudofolliculitis
baibae, iosacea, peiioial deimatitis.
Iruok Ma|asse:ía folliculitis, °hot-tub" pseu-
domonas folliculitis, S. aureus folliculitis, and
acne-like conditions (see above).
IA80kAI0k¥ £XAMINAII0N
No laboiatoiy examinations iequiied. If theie
is suspicion of an endociine disoidei, fiee
testosteione, follicle-stimulating hoimone,
luteinizing hoimone, and DHEAS should be
deteimined to exclude hypeiandiogenism and
polycystic ovaiy syndiome. Noìe : In the ovei-
whelming majoiity of acne patients, hoimone
levels aie noimal.
Laboiatoiy examinations ¦tiansaminases
(ALT, AST), tiiglyceiides, and cholesteiol levels]
may be iequiied if systemic isotietinoin tieat-
ment is planned (see below).
C0ükS£
Acne most often cleais spontaneously by the
eaily twenties but can peisist to the fouith
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' T
Oial isotietinoin leads to complete iemission in
almost all cases, which last foi months to yeais
in the majoiity of patients.
1ndIcutIvns ]vr Oru| 1svtretInvIn Foi modei-
ate and seveie, iecalcitiant, nodulai acne. The
patient must have been iesistant to othei acne
theiapies, including systemic antibiotics.
CvntruIndIcutIvns Isotietinoin is teiatogenic.
Theiefoie, piegnancy must be pievented and ef-
fective contiaception is necessaiy, i.e., oial. Both
tetiacycline and isotietinoin may cause pseudo-
tumoi ceiebii (benign intiacianial swelling);
theiefoie, the two medications should ne·er be
used togethei.
WurnIngs Blood lipids and tiansaminases
(ALT, AST) should be deteimined befoie thei-
apy. About 25% of patients can develop ín-
treaseJ ¡|asma ìríg|yteríJes ; 15% of patients a
deciease in |íg|-Jensíìy |í¡o¡roìeíns , and about
7% an íntrease ín t|o|esìero| |e·e|s . This may
inciease the caidiovasculai iisk. When levels of
seium tiiglyceiides iise above 800 mg/µL, the
patient may develop acute pancieatitis. Patients
should not take vitamin supplements contain-
ing vitamin A. He¡aìoìoxítíìy has been veiy
iaiely iepoited in the foim of clinical hepatitis,
but patients may develop mild to modeiate
elevation of tiansaminase levels that noimalize
FICük£ 1-4 Acoe coo¿|obata |u |||º ºe.e|e uodu|oc,º||c +cue, ||e|e +|e |+|çe cou||ueu| uodu|eº +ud c,º|º
|o|r|uç ||ue+| rouudº ||+| co||eºpoud |o |u|e|couuec||uç c|+uue|º. I|e|e |º poº|u|+||ou, ºc+|||uç +ud |eº|ouº +|e
.e|, p+|u|u|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 8
with ieduction of the dose of the diug. Eyes.
níg|ì ||ínJness has been iepoited, and patients
should be wained about diiving at night. Also,
patients may have JetreaseJ ìo|erante ìo tonìatì
|enses duiing and aftei theiapy. S|ín : an eczema-
like iash due to diug-induced diyness often
appeais, and this iesponds diamatically to low
potency (class III) topical glucocoiticoids. Diy
lips and cheilitis occui in piactically all patients
and must be tieated. Reveisible thinning of haii
may occui veiy iaiely, as may paionychia. Nose :
diyness of nasal mucosa and nose bleeds (iaie).
Oì|er sysìems : iaiely, depiession, headaches,
aithiitis, and musculai pain. Foi additional
iaie possible complications, consult the pack-
age inseit.
Dvsuge Isotietinoin, 0.5 to 1 mg/kg given in
divided doses with food. Most patients impiove
and cleai within 20 weeks with 1 mg/kg. Foi
seveie disease, especially on the tiunk, 2 mg/kg
and longei tieatment may be iequiied. As many
as thiee oi moie couises of isotietinoin have
been given in iefiactoiy cases, but in most cases
a single couise is sufficient to induce lasting
iemission.
0ther Systemìc Ireatmeots Ior Severe Acoe
Systemic glucocoiticoids may be iequiied
in seveie acne conglobata, acne fulminans,
and the SAPHO and PAPA syndiomes. The
TNF- inhibitoi infliximab and anakinia aie
investigational diugs in these seveie foims and
show piomising effects. Noìe : Foi inflammatoiy
cysts and nodules, intialesional tiiamcinolone
(0.05 mL of a 3 to 5 mg/mL solution) is indi-
cated. Website: |ìì¡.//www.aaJ.org/¡am¡||eìs/
atne¡am¡.|ìm|
FICük£ 1-5 Acoe coo¿|obata |u||+rr+|o|, uodu|eº +ud c,º|º |+.e co+|eºced, |o|r|uç +|ºceººeº +ud e.eu
|e+d|uç |o u|ce|+||ou. I|e|e +|e ru|||p|e coredoueº +ud r+u, |eceu| |ed ºc+|º |o||oW|uç |eºo|u||ou o| |u||+rr+|o|,
|eº|ouº ou ||e uppe| c|eº|, uec|, +ud +|rº.
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 9
A corrou c||ou|c |u||+rr+|o|, +cue||o|r d|ºo|·
de| o| ||e |+c|+| p||oºe|+ceouº uu||º.
|| |º coup|ed W||| +u |uc|e+ºed |e+c||.||, o| c+p||·
|+||eº |e+d|uç |o ||uº||uç +ud |e|+uç|ec|+º|+.
\+, |eºu|| |u |u||e|, |||c|eu|uç o| uoºe, c|ee|º,
|o|e|e+d, o| c||u due |o ºe|+ceouº |,pe|p|+º|+,
eder+, +ud ||||oº|º
k0SAC£A lCD-9: o95.¯

lCD-10: L71
£FI0£MI0I0C¥
0ccurreoce Common, affecting appioximately
10% of faii-skinned people.
A¿e oI 0oset 30 to 50 yeais; peak incidence
between 40 and 50 yeais.
Sex Females piedominantly, but ihinophyma
occuis mostly in males.
kace Celtic peisons (skin phototypes I and II)
but also southein Mediteiianeans; less fiequent
oi iaie in pigmented peisons (skin phototypes
V and VI, i.e., biown and black)
SIACINC (FI£WIC AN0 kIICMAN
CIASSIFICAII0N)
T|e rosatea Jíaì|esís : episodic eiythema,
°flushing and blushing"
Sìage I. Peisistent eiythema with tel-
angiectases
Sìage II. Peisistent eiythema, telangiectases,
papules, tiny pustules.
Sìage III. Peisistent deep eiythema, dense
telangiectases, papules, pustules, nodules;
iaiely peisistent °solid" edema of the cen-
tial pait of the face
Noìe. piogiession fiom one stage to anothei
does not always occui. Rosacea may stait with
stage II oi III and stages may oveilap.
CIINICAI MANIF£SIAII0N
Usually a histoiy of episodic ieddening of the
face (flushing) with incieases in skin tempeia-
tuie in iesponse to heat stimuli in the mouth
(hot liquids); spicy foods; alcohol,. Exposuie
to sun-iosacea is often associated with solai
elastosis-and heat (such as chefs woiking neai
a hot stove) may cause exaceibations. Acne may
have pieceded the onset of iosacea by yeais;
neveitheless, iosacea may and usually does aiise
de novo without any pieceding histoiy of acne
oi seboiihea.
0uratìoo oI Iesìoos Days, weeks, months.
Skìo Symptoms Concein about cosmetic facial
appeaiance; patients aie often peiceived as be-
ing alcoholic-which, of couise, is not tiue.
Skìo Iesìoos Eur|y Pathognomonic flush-
ing- °ied face" (Fig. 1-6); tiny papules and
papulopustules (2-3 mm), pustule often
small ( 1 mm) and on the apex of the papule
(Figs. 1-7 and 1-8). No tomeJones .
Lute Red facies and dusky-ied papules and
nodules (Figs. 1-6 to 1-9) Scatteied, disciete
lesions. Telangiectases. Maiked sebaceous hy-
peiplasia and lymphedema in chionic iosacea,
causing disfiguiement of the nose, foiehead,
eyelids, eais, and chin.
DIstrIhutIvn Chaiacteiistic is the symmetiic
localization on the face (Fig. 1-7). Raiely, neck,
chest (V-shaped aiea), back, and scalp.
Specìa| Iesìoos
R|íno¡|yma (enlaiged nose), meìo¡|yma (en-
laiged cushion-like swelling of the foiehead),
||e¡|aro¡|yma (swelling of the eyelids), oìo-
¡|yma (cauliflowei-like swelling of the eai-
lobes), and gnaì|o¡|yma (swelling of the chin)
iesult fiom maiked sebaceous gland hypeipla-
sia (Fig. 1-11) and fibiosis. Upon palpation:
soft, iubbei-like.
£ye Iovo|vemeot
°Red" eyes as a iesult of chionic blephaiitis,
conjunctivitis, and episcleiitis. Rosacea keiatitis,
albeit iaie, is a seiious pioblem because coineal
ulceis may develop.
IA80kAI0k¥ £XAMINAII0NS
8acterìa| Cu|ture Rule out S. aureus infection.
Sciapings may ieveal massive concuiient
DemoJex [o||ítu|orum infestation.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 10
0ermatopatho|o¿y Nonspecific peiifollicu-
lai and peiicapillaiy inflammation with oc-
casional foci of °tubeiculoid" gianulomatous
aieas; dilated capillaiies. Foci of neutiophils
high and within the follicle. Laìer sìages : dif-
fuse hypeitiophy of the connective tissue,
sebaceous gland hypeiplasia, epithelioid gian-
uloma without caseation, and foieign-body
giant cells.
RhInvphymu Veiy maiked lobulai sebaceous hy-
peiplasia ( g|anJu|ar ìy¡e ) and/oi maiked inciease
in connective tissue ( [í|rous ìy¡e ) with laige ec-
tatic veins ( [í|roangíomaìous ìy¡e ).
0IFF£k£NIIAI 0IACN0SIS
Facìa| Fapu|es[Fustu|es Acne (in iosacea
theie aie no comedones), peiioial deimatitis,
S. aureus folliculitis, giam-negative folliculitis,
D. [o||ítu|orum infestation.
Facìa| F|ushìo¿[£rythema Seboiiheic deimati-
tis, piolonged use of topical glucocoiticoids, sys-
temic lupus eiythematosus; deimatomy ositis.
C0ükS£
Fro|oo¿ed Recuiiences aie common. Aftei a
few yeais, the disease may disappeai spontane-
ously; usually it is foi life time. Men and veiy
iaiely women may develop ihinophyma.
MANAC£M£NI
Freveotìoo Maiked ieduction oi elimination
of alcohol may be helpful in some patients.
FICük£ 1-6 £rythematous rosacea (sta¿e I) I|e e+||, º|+çeº o| |oº+ce+ o||eu p|eºeu| |, ep|ºod|c e|,·
||er+, '||uº||uç +ud ||uº||uç,¨ W||c| |º |o||oWed |, pe|º|º|eu| e|,||er+, W||c| |º due |o ru|||p|e ||u, |e|+uç|ec·
|+º|+º, |eºu|||uç |u + |ed |+ce.
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 11
Iopìca|
MeìroníJa:o|e ge| oi tream , 0.75%, twice
daily
MeìroníJa:o|e tream , 1%, once daily
SoJíum su|[ateìamíJe, su|[ur |oìíons 10% and
5%
To¡íta| anìí|íoìíts (e.g., eiythiomycin gel) aie
less effective.
Systemìc Oial antibiotics aie moie effective
than topical tieatment.
Mínotyt|íne or Joxytyt|íne , 50-100 mg twice
daily, fiist-line antibiotics; veiy effective
(doxycycline is a phototoxic diug and its
use limits exposuie to sunlight in sum-
mei).
Teìratyt|íne , 1-1.5 g/d in divided doses until
cleai; then giadually ieduce to once-daily
doses of 250-500 mg, most effective is oial
metionidazole 500 mg BID.
A dose of 50 mg minocycline oi doxycycline
oi 250-500 g tetiacycline is given as mainte-
nance.
0ra| Isotretìooìo Foi individuals with seveie
disease (especially stage III) not iesponding to
antibiotics and topical tieatments. A low-dose
iegimen of 0.1-0.5 mg/kg body weight pei day
is effective in most patients, but occasionally
1 mg/kg may be iequiied.
Ivermectìo 12 mg PO in case of massive de-
modex infestation.
khìoophyma aod Ie|ao¿ìectasìa Tieated by
suigeiy oi lasei suigeiy with excellent cosmetic
iesults. Website |ìì¡.//www.aaJ.org/¡am¡||eìs/
rosatea.|ìm|
FICük£ 1-T kosacea \ode|+|e|, ºe.e|e |oº+ce+ |u + 29·,e+|·o|d |er+|e W||| pe|º|º|eu| e|,||er+,
|e|+uç|ec|+º|+, |ed p+pu|eº (º|+çe ||), +ud ||u, puº|u|eº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 12
FICük£ 1-8 kosacea, sta¿es II-III Ie|+uç|ec|+º|+, p+pu|eº +ud puº|u|eº, +ud ºore ºWe|||uç |u + 50·,e+|·o|d
Wor+u. I|e|e +|e uo coredoueº.
FICük£ 1-9 Fapu|opustu|ar rosacea (traosìtìoo oI sta¿e II to sta¿e III) |u |||º o5·,e+|·o|d |er+|e, |oº+·
ce+ |u.o|.eº +|roº| ||e eu|||e |+ce, ºp+||uç ou|, ||e uppe| ||p +ud c||u. |+pu|eº +ud puº|u|eº |+.e co+|eºced-+ç+|u
uo coredoueº-+ud |+.e +||e+d, |ed |o ºore ºWe|||uç o| ||e c|ee|º, W||c| p|eºeu| 'ºo||d¨ eder+.
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 13
FICük£ 1-10 kosacea, traosìtìoo oI sta¿e II
to III \u|||p|e |||ç|| |ed p+pu|eº +ud puº|u|eº
+ud ºore ºWe|||uç o| ||e ||ç|| c|ee| o| + 52·
,e+|·o|d Wor+u. |o|e ||+| |u |||º c+ºe |eº|ouº +|e
c|uº|e|ed, ||e |o|e|e+d |º ||ee o| |e|+uç|ec|+º|+,
+ud ||e |eº|ouº +|e uo| +|ºo|u|e|, º,rre|||c.
FICük£ 1-11 kosacea (sta¿e III) ne|e ||e pe|º|º|eu| 'ºo||d¨ eder+ o| ||e uoºe, |o|e|e+d, +ud p+||º o| ||e
c|ee|º |º ||e |e+d|uç º,rp|or. |+pu|eº, puº|u|eº, +ud c|uº|ed puº|u|eº +|e ºupe||rpoºed ou |||º pe|º|º|eu| eder+.
I|e eu|+|çed uoºe |ee|º |u||e|, +ud +||e+d, |ep|eºeu|º |||uop|,r+.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 14
||ºc|e|e e|,||er+|ouº r|c|op+pu|eº +ud r|·
c|o.eº|c|eº,
0||eu cou||ueu| |u ||e pe||o|+| +ud pe||o||||+|
º||u
0ccu|º r+|u|, |u ,ouuç Woreu, c+u occu| |u
c|||d|eu +ud ||e o|d
k+|e|,
F£kI0kAI 0£kMAIIIIS lCD-9: o95.¯

lCD-10: L71.0
*
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset 16-45 yeais; can occui in chil-
dien and the old.
Sex Females piedominantly.
£tìo|o¿y Unknown but may be maikedly ag-
giavated by potent topical (fluoiinated) gluco-
coiticoids.
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos Weeks to months. Skin
symptoms peiceived as cosmetic disfiguiement;
occasional itching oi buining, feeling of tight-
ness.
Skìo Iesìoos 1- to 2-mm eiythematous pa-
pulopustules on an eiythematous backgiound
(Fig. 1-12) iiiegulaily giouped, symmet-
iic. Lesions inciease in numbei with cential
confluence and satellites; confluent plaques may
appeai eczematous with tiny scales. Theie aie
no comedones.
DIstrIhutIvn Initially peiioial. Rim of spaiing
aiound the veimilion boidei of lips (Fig. 1-12).
At times, in the peiioibital aiea (Figs. 1-13, 1-14).
Uncommonly, only peiioibital involvement; oc-
casionally, glabella and foiehead. Commonly on
the moustache aiea and lateial chin.
IA80kAI0k¥ £XAMINAII0NS
Cu|ture Rule out S. aureus infection.
0IFF£k£NIIAI 0IACN0SIS
Alleigic contact deimatitis, atopic deimatitis,
seboiiheic deimatitis, iosacea, acne vulgaiis,
steioid acne.
FICük£ 1-12 Ferìora| dermatìtìs \ode|+|e |u.o|.ereu| W||| e+||, cou||ueuce o| ||u, p+pu|eº +ud + |eW
puº|u|eº |u + pe||o|+| d|º||||u||ou |u + ,ouuç Wor+u. |o|e |,p|c+| ºp+||uç o| ||e .e|r|||ou |o|de| (rucocu|+ueouº
juuc||ou).
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 15
C0ükS£
Appeaiance of lesions usually subacute ovei
weeks to months. At times, misdiagnosed as
an eczematous oi a seboiiheic deimatitis and
tieated with a potent topical glucocoiticoid
piepaiation, aggiavating peiioial deimatitis oi
inducing steioid acne. Untieated, peiioial dei-
matitis fluctuates in activity ovei months to
yeais but is not neaily as chionic as iosacea.
MANAC£M£NI
Iopìca|
Avoid topical glucocoiticoids!
MeìroníJa:o|e , 0.75% gel two times daily oi
1% once daily
Eryì|romytín , 2% gel applied twice daily
Systemìc
Mínotyt|íne oi Joxytyt|íne , 100 mg daily
until cleai, then 50 mg daily foi anothei 2
months (caution, doxycycline is a photo-
sensitizing diug) oi
Teìratyt|íne , 500 mg twice daily until cleai,
then 500 mg daily foi 1 month, then 250
mg daily foi an additional month.
FICük£ 1-13 Ferìora| dermatìtìs ||e|e|eu||+| |oc+·
||ou ou ||e c||u |u| +|ºo ou ||e |oWe| e,e||dº |u + o+·,e+|·
o|d Wor+u. A| |||º +çe, d|||e|eu||+| d|+çuoº|º |uc|udeº
|oº+ce+, |u| || Wou|d |e uuuºu+| |o| |oº+ce+ |o |u.o|.e
||e pe||o|+| |eç|ou +ud e,e||dº |u| ºp+||uç ||e c|ee|º
+ud uoºe.
FICük£ 1-14 Ferìorbìta| dermatìtìs |o|e p|eºeuce o| ||u, p+pu|eº +ud + |eW puº|u|eº +|ouud ||e e,e. I||º
|º + ruc| |eºº corrou º||e ||+u ||e |eº|ouº +|ouud ||e rou||.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 16
A c||ou|c, ºuppu|+||.e, o||eu c|c+|||c|+| d|ºe+ºe o|
+poc||ue ç|+ud-|e+||uç º||u.
|u.o|.eº ||e +\|||+e, ||e +uoçeu||+| |eç|ou,
+ud, |+|e|,, ||e ºc+|p (c+||ed :·:c|··c·¤¸ ¤-··|¤|·
|·:±|·|·º).
\+, |e +ººoc|+|ed W||| ºe.e|e uodu|oc,º||c +cue
+ud p||ou|d+| º|uuºeº (|e|red |¤||·:±|c· ¤::|±º·¤¤
º,¤!·¤¤-).
',¤¤¤,¤º. Apoc||u|||º, ||d|+deu|||º +\|||+||º,
+|ºceºº o| ||e +poc||ue ºWe+| ç|+udº.
hI0kA0£NIIIS SüFFükAIIvA |C|·9. 105.3!

|C|·¹0. |1!.2
£FI0£MI0I0C¥
A¿e oI 0oset Fiom pubeity to climacteiic.
Sex Affects moie females than males; esti-
mated to be 4% of female population. Males
moie often have anogenital and females axillaiy
involvement.
kace All iaces.
heredìty Mothei-daughtei tiansmission has
been obseived. Families give a histoiy of nod-
ulocystic acne and hidiadenitis suppuiativa
occuiiing sepaiately oi togethei in blood iela-
tives.
£II0I0C¥ AN0 FAIh0C£N£SIS
Unknown. Piedisposing factois: obesity, genetic
piedisposition to acne, folliculai plugging of
apociine iegions, secondaiy bacteiial infection.
FAIh0C£N£SIS
The following sequence may be the mechanism
of the development of the lesions: keiatinous
plugging of the haii follicle dilatation haii
follicle and secondaiily of the apociine duct
inflammatoiy changes limited to a single
apociine gland bacteiial giowth in dilated
follicle and duct iuptuie iesulting in exten-
sion of inflammation/infection extension of
suppuiation/tissue destiuction ulceiation
and fibiosis, sinus tiact foimation.
CIINICAI MANIF£SIAII0N
Sym¡ìoms : Inteimittent pain and maiked point
tendeiness ielated to abscess.
Skìo Iesìoos Initial lesion: ·ery ìenJer , ied
inflammatoiy nodule/abscess (Fig. 1-15) that
may iesolve oi diain puiulent/seiopuiulent
mateiial. The same lesion may appeai iepeatedly
in the same location. Open comedones, and
at times unique Jou||e comedones, aie highly
chaiacteiistic (Fig. 1-15), may be piesent even
when active nodules aie absent. Eventually,
modeiately to exquisitely tendei sinus tiacts may
foim. Pus diains fiom opening of abscess and
sinus tiacts; fibiosis, °biidge" scais, hypeitiophic
and keloidal scais, contiactuies foim (Figs. 1-16,
1-17). Raiely, lymphedema of the associated
limb may develop.
DIstrIhutIvn Axillae, bieasts, anogenital aiea,
gioin. Often bilateial in axillae and/oi ano-
genital aiea; may extend ovei entiie back, but-
tocks, peiineum involving sciotum oi vulva
(Fig. 1-18), and scalp.
AssvcIuted FIndIngs Cystic acne, pilonidal
sinus. Often obesity.
IA80kAI0k¥ £XAMINAII0NS
8acterìo|o¿y Vaiious pathogens may second-
aiily colonize oi °infect" lesions. These include
S. aureus , stieptococci, Est|erít|ía to|í, Proìeus
míra|í|ís , and PseuJomonas aerugínosa .
0ermatopatho|o¿y Ear|y : keiatin occlusion of
haii follicle, ductal/tubulai dilatation, inflam-
matoiy changes limited to folliculai appaiatus.
Laìe : destiuction of apociine/ecciine/piloseba-
ceous appaiatus, fibiosis, pseudoepithelioma-
tous hypeiplasia in sinuses.
0IFF£k£NIIAI 0IACN0SIS
Painful papule, nodule, abscess in gioin and
axilla. Ear|y : fuiuncle, caibuncle, lymphadeni-
tis, iuptuied inclusion cyst, painful lymphad-
enopathy in lymphogianuloma veneieum oi
cat-sciatch disease. Laìe : lymphogianuloma
veneieum, donovanosis, sciofulodeima, actino-
mycosis, sinus tiacts and fistulas associated with
ulceiative colitis and iegional enteiitis.
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 1T
FICük£ 1-15 hìdradeoìtìs sup-
puratìva \+u, ||+c| coredoueº,
ºore o| W||c| +|e p+||ed, +|e + c|+|·
+c|e||º||c ||ud|uç, +ººoc|+|ed W||| deep,
e\qu|º||e|, p+|u|u| +|ºceººeº +ud o|d
ºc+|º |u ||e +\|||+.
FICük£ 1-16 hìdradeoìtìs suppuratìva \u|||p|e |u|ç|uç +ud dep|eººed ºc+|º puc|e||uç ||e ºu||ouud|uç
º||u +ud d|+|u|uç º|uuºeº |u ||e +\|||+ o| + 22·,e+|·o|d |er+|e.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 18
C0ükS£ AN0 Fk0CN0SIS
The seveiity of the disease vaiies consideiably.
Many patients have only mild involvement with
iecuiient, self-healing, tendei ied nodules and
do not seek theiapy. The disease usually un-
deigoes a spontaneous iemission with age
(35 yeais). In some individuals, the couise
can be ielentlessly piogiessive, with maiked
moibidity ielated to chionic pain, diaining
sinuses, and scaiiing, with iestiicted mobility
(Fig. 1-18) . Complications (iaie): fistulas
to uiethia, bladdei, and/oi iectum; anemia,
amyloidosis.
MANAC£M£NI
Hidiadenitis suppuiativa is noì simply an infec-
tion, and systemic antibiotics aie only pait of
the tieatment piogiam. Combinations of (1)
intialesional glucocoiticoids, (2) suigeiy, (3)
oial antibiotics, and (4) isotietinoin aie used.
Medìca| Maoa¿emeot
Acute FaìoIu| Iesìoos Nvdu|e Intialesional tii-
amcinolone (3-5 mg/mL).
Ahscess Intialesional tiiamcinolone (3-5 mg/
mL) into the wall followed by incision and
diainage of abscess fluid.
Chrooìc Iow-Crade 0ìsease Oial antibiotics:
eiythiomycin (250 - 500 mg qid), tetiacycline
(250-500 mg qid), oi minocycline (100 mg
twice daily) until lesions iesolve, oi a combina-
tion of clindamycin twice daily 300 mg bid with
iifampin (300 mg twice daily); may take weeks.
Fredoìsooe May be given concuiiently if pain
and inflammation aie seveie: 70 mg daily foi 2
to 3 days, tapeied ovei 14 days.
0ra| Isotretìooìo Not useful in seveie disease,
but useful in eaily disease to pievent folliculai
plugging and when combined with suigical ex-
cision of individual lesions.
Sur¿ìca| Maoa¿emeot
· Incise and diain acute abscesses.
· Excise chionic iecuiient, fibiotic nodules oi
sinus tiacts. If one oi two nodules can be
pinpointed with iecuiient disease, they can
be excised with a good iesult.
· With extensive, chionic disease, complete
excision of axilla oi involved anogenital aiea
may be iequiied. Excision should extend
down to fascia and iequiies split skin giafting.
Fsycho|o¿ìca| Maoa¿emeot
These patients need constant ieassuiance, as
they become veiy depiessed because of the na-
tuie of the illness, e.g., pain, soiling of clothing
by diaining pus, odoi, and the site of occui-
ience (anogenital aiea). Theiefoie, eveiy effoit
should be made to deal with the disease, using
eveiy modality possible.
S£CII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 19
FICük£ 1-1T hìdradeoìtìs sup-
puratìva 'e.e|e ºc+|||uç ou ||e
|u||oc|º, |u||+rr+|o|, p+|u|u| uod·
u|eº W||| ||º|u|+º +ud d|+|u|uç º|uuºeº.
w|eu ||e p+||eu| º||º doWu, puº W|||
ºqu||| ||or ||e º|uuº opeu|uçº.
FICük£ 1-18 hìdradeoìtìs suppuratìva I|e eu|||e pe||çeu||+| +ud pe||+u+| º||u +º We|| +º ||e |u||oc|º +ud
|uue| +ºpec|º o| ||e |||ç|º +|e |u.o|.ed |u |||º 50·,e+|·o|d r+|e. I|e|e |º couº|de|+||e |u||+rr+||ou, +ud p|eººu|e
|e|e+ºeº pu|u|eu| e\ud+|e ||or ru|||p|e º|uuºeº. I|e p+||eu| |+d |o We+| + |+|çe d|+pe|, |ec+uºe W|eue.e| |e W+º
ºe+|ed, ºec|e||ouº Wou|d ºqu||| ||or ||e º|uuºeº.
20
S E C I | 0 N 2
£CI£MA[0£kMAIIIIS
I|e |e|rº -:c-¤c +ud !-·¤c|·|·º +|e uºed
|u|e|c|+uçe+||,, deuo||uç + po|,ro|p||c |u||+r·
r+|o|, |e+c||ou p+||e|u |u.o|.|uç ||e ep|de|r|º
+ud de|r|º. I|e|e +|e r+u, e||o|oç|eº +ud + W|de
|+uçe o| c||u|c+| ||ud|uçº. Acu|e ec/er+/de|r+||||º
|º c|+|+c|e||/ed |, p|u|||uº, e|,||er+, +ud .e·
º|cu|+||ou, c||ou|c ec/er+/de|r+||||º, |, p|u|||uº,
\e|oº|º, ||c|eu|||c+||ou, |,pe||e|+|oº|º,  ||ººu||uç.
|C| |º + |oc+||/ed d|ºe+ºe cou||ued |o +|e+º e\·
poºed |o |||||+u|º.
|| |º c+uºed |, e\poºu|e o| ||e º||u |o c|er|c+| o|
o||e| p|,º|c+| +çeu|º ||+| +|e c+p+||e o| |||||+||uç
||e º||u, +cu|e|, o| c||ou|c+||,.
'e.e|e |||||+u|º c+uºe |o\|c |e+c||ouº e.eu +||e| +
º|o|| e\poºu|e.
\oº| c+ºeº +|e c+uºed |, c||ou|c curu|+||.e
e\poºu|e |o oue o| ro|e |||||+u|º.
I|e |+udº +|e ||e roº| corrou|, +||ec|ed +|e+.
|u +dd|||ou |o de|r+||||º, |||||+u| cou|+c| |eºpouºeº
o| ||e º||u |uc|ude. ºu|jec||.e |||||+uc,, ||+uº|eu|
|||||+u| |e+c||ouº, pe|º|º|eu| |||||+u| |e+c||ouº, |o\|c
(c+uº||c) |u|u.
|||||+u| cou|+c| |eºpouºeº o| º||u +ppeud+çeº
+ud p|çreu|+|, º,º|er |uc|ude. |o|||cu|+| +ud
+cue||o|r e|up||ouº, r|||+||+, p|çreu|+|, c|+uçeº
(|,po· +ud |,pe|p|çreu|+||ou), ç|+uu|or+|ouº
|e+c||ouº, +ud +|opec|+.
IkkIIANI C0NIACI 0£kMAIIIIS (IC0)
|C|·9 . o92.9

|C|·¹0 . |2+ 
C¤¤|c:| !-·¤c|·|·º |º + çeue||c |e|r +pp||ed |o
+cu|e o| c||ou|c |u||+rr+|o|, |e+c||ouº |o ºu|·
º|+uceº ||+| core |u cou|+c| W||| ||e º||u. |||||+u|
cou|+c| de|r+||||º (|C|) |º c+uºed |, + c|er|c+|
|||||+u|, +||e|ç|c cou|+c| de|r+||||º (AC|) |, +u +u·
||çeu (+||e|çeu) ||+| e||c||º + |,pe |\ (ce||·red|+|ed
o| de|+,ed) |,pe|ºeuº|||.||, |e+c||ou.
I|e +cu|e |o|r o| |C| occu|º +||e| + º|uç|e
e\poºu|e |o ||e o||eud|uç +çeu| ||+| |º |o\|c |o ||e
º||u (e.ç., c|o|ou o||, p|euo|º, |e|oºeue, o|ç+u|c
ºo|.eu|º, ºod|ur +ud po|+ºº|ur |,d|o\|de, ||re
+c|dº) +ud |u ºe.e|e c+ºeº r+, |e+d |o uec|oº|º.
|| |º depeudeu| ou couceu||+||ou o| ||e o||eud|uç
+çeu| +ud occu|º |u e.e|,oue, depeud|uç ou
||e peue||+|||||, +ud |||c|ueºº o| ||e º||+|ur
co|ueur. I|e|e |º + |||eº|o|d couceu||+||ou |o|
||eºe ºu|º|+uceº +|o.e W||c| ||e, c+uºe +cu|e
de|r+||||º +ud |e|oW W||c| ||e, do uo|. I||º
ºe|º +cu|e |C| +p+|| ||or +cu|e AC|, W||c|
|º depeudeu| ou ºeuº|||/+||ou +ud ||uº occu|º
ou|, |u ºeuº|||/ed |ud|.|du+|º. |epeud|uç ou ||e
deç|ee o| ºeuº|||/+||ou, r|uu|e +rouu|º o| ||e
o||eud|uç +çeu|º r+, e||c|| + |e+c||ou. '|uce |C| |º
+ |o\|c p|euoreuou, || |º cou||ued |o ||e +|e+ o|
e\poºu|e +ud |º ||e|e|o|e +|W+,º º|+|p|, r+|ç|u·
+|ed +ud ue.e| ºp|e+dº. AC| |º +u |rruuo|oç|c
|e+c||ou ||+| |eudº |o |u.o|.e ||e ºu||ouud|uç
º||u (ºp|e+d|uç p|euoreuou) +ud r+, e.eu
ºp|e+d |e,oud +||ec|ed º||eº. Ceue|+||/+||ou r+,
occu|.
C0NIACI 0£kMAIIIIS |C|·9 . o92·9

|C|·¹0 . |25
S£CII0N 2 EC/E\A/|Ek\AI|I|' 21
IA8I£ 2-1 Nost Common |rritant/Io\ic A¿ents
· 'o+pº, de|e|çeu|º, W+|e||eºº |+ud c|e+ue|º
· Ac|dº +ud +||+||º*. |,d|o||uo||c +c|d, cereu|, c||or|c +c|d, p|oºp|o|uº, e||,|eue o\|de, p|euo|, re|+| º+||º.
· |uduº|||+| ºo|.eu|º. co+| |+| ºo|.eu|º, pe||o|eur, c||o||u+|ed |,d|oc+||ouº, +|co|o| ºo|.eu|º, e||,|eue ç|,co| e||e|,
|u|peu||ue, e||,| e||e|, +ce|oue, c+||ou d|o\|de, |\'0, d|o\+ue, º|,|eue.
· ||+u|º. Eup|o|||+ce+e (ºpu|çeº, c|o|ouº, po|uºe|||+º, r+c|uee| ||ee). k+cuucu|+ce+e (|u||e|cup), C|uc||e|+e (||+c|
ruº|+|d), u|||c+ce+e (ue|||eº), 'o|+u+ce+e (peppe|, c+pº+|c|u), 0puu||+ (p||c||, pe+|).
· 0||e|º. |||e|ç|+ºº, Woo|, |ouç| º,u||e||c c|o|||uç, |||e·|e|+|d+u| |+|||cº, '|Ck¨ p+pe|.
*
|e+d |o c|er|c+| |u|uº +ud uec|oº|º, || couceu||+|ed.
£FI0£MI0I0C¥
ICD is the most common foim of occupational
skin disease, accounting foi up to 80% of all oc-
cupational skin disoideis. Howevei, ICD need
not be occupational and can occui in anyone
being exposed to a substance iiiitant oi toxic
to the skin.
0ccupatìooa| £xposure Individuals engaged in
the following occupations/activities aie at iisk
foi ICD: housekeeping; haiidiessing; medical,
dental, and veteiinaiy seivices; cleaning; floial
aiianging; agiicultuie; hoiticultuie; foiestiy;
food piepaiation and cateiing; piinting; paint-
ing; metal woik; mechanical engineeiing; cai
maintenance; constiuction; fishing.
£II0I0C¥
£tìo|o¿ìc A¿eots (Table 2-1) Abiasives, clean-
ing agents, oxidizing agents (e.g., sodium hy-
pochloiite); ieducing agents, plants and animal
enzymes, secietions; dessicant powdeis, dust,
soils; excessive exposuie to watei.
Fredìsposìo¿ Factors Atopics with a histoiy
of atopic deimatitis aie at highest iisk foi
ICD; the majoiity of woikeis with significant
occupational ICD aie atopics. Otheis: white
skin, tempeiatuie (low), climate (low humid-
ity), occlusion, mechanical iiiitation. Cement
ICD tends to flaie in summei in hot humid
climates.
FAIh0C£N£SIS
Iiiitants (both chemical and physical), cause
cell damage if applied foi sufficient time and
in adequate concentiation. ICD occuis when
defense oi iepaii capacity of the skin is unable
to maintain noimal skin integiity and function
oi when penetiation of chemical(s) induces an
inflammatoiy iesponse. Lessei iiiitants cause
ieaction only aftei piolonged exposuie. The
initial ieaction is usually limited to the site of
contact with the iiiitant; the concentiation of
iiiitant diffusing outside the aiea of contact
almost always falls below the ciitical thieshold
necessaiy to piovoke a ieaction.
Mechanisms involved in acute and chionic
phases of ICD aie fundamentally diffeient.
Acute ieactions involve diiect cytotoxic dam-
age to keiatinocytes. Chionic ICD iesults fiom
iepeated exposuies that cause slow damage to
cell membianes, disiupting the skin baiiiei and
leading to piotein denatuiation and cellulai
toxicity.
ACüI£ IkkIIANI C0NIACI 0£kMAIIIIS
CIINICAI MANIF£SIAII0N
Symptoms In some individuals, subjective
symptoms (buining, stinging, smaiting) may
be the only manifestations. Painful sensations
can occui within seconds aftei exposuie
(immediate-type stinging), e.g., to acids,
chloiofoim, and methanol. Delayed-type
stinging occuis within 1 to 2 min, peaking at
5 to 10 min, fading by 30 min, and is caused
by agents such as aluminum chloiide, phenol,
piopylene glycol, and otheis. In acute delayed
ICD, objective skin symptoms do not stait
until 8-24 h aftei exposuie (e.g., anthialin,
ethylene oxide, benzalkonium chloiide) and aie
accompanied by buining iathei then itching.
Skìo Fìodìo¿s May occui minutes aftei
exposuie oi may be delayed up to  24 h. The
spectium of changes ianges fiom eiythema
to vesiculation (Figs. 2-1 and 2-2) and caustic
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 22
buin with neciosis. Acute ICD iepiesents
shaiply demaicated eiythema and supeificial
edema, coiiesponding to the application site
of the toxic substance (Fig. 2-1). Lesions do
not spiead beyond the site of contact. In moie
seveie ieactions vesicles and blisteis aiise
within the eiythematous lesions (Figs. 2-1 and
2-2), followed by eiosions and/oi even fiank
neciosis, as with acids oi alkaline solutions.
No papules. Configuiation often bizaiie
oi lineai (°outside job" oi diipping effect)
(Fig. 2-1).
£vo|utìoo oI Iesìoos Eiythema with a dull,
nonglistening suiface (Fig. 2-1) vesicula-
tion (oi blistei foimation) (Figs. 2-1 and 2-2)
eiosion ciusting shedding of ciusts
and scaling oi (in chemical buin) eiythema
neciosis shedding of neciotic tissue
ulceiation healing.
DIstrIhutIvn Isolated, localized to one iegion
oi geneialized (plant deimatitis), depending on
contact with toxic agent.
DurutIvn Days, weeks depending on tissue
damage.
Coostìtutìooa| Symptoms
Usually none, but in widespiead acute ICD
°acute illness" syndiome, fevei may occui.
Chk0NIC IkkIIANI C0NIACI
0£kMAIIIIS
I¥F£S
Cumu|atìve IC0 Most common; develops
slowly aftei iepeated additive exposuie to mild
iiiitants (watei, soap, deteigents, etc.), usu-
ally on hands. Repeated exposuies to toxic oi
subtoxic concentiations of offending agents
usually associated with a chionic distuibance of
the baiiiei function that allows even subtoxic
concentiations of offending agents to penetiate
into the skin and elicit a chionic inflamma-
toiy iesponse; e.g., aftei iepeated exposuie
to alkaline deteigents and oiganic solvents,
which, if applied only once to noimal skin, do
not elicit a ieaction. Injuiy (e.g., iepeated iub-
bing of the skin), piolonged soaking in watei,
oi chionic contact aftei iepeated, cumulative
physical tiauma-fiiction, piessuie, abiasions
in individuals engaged in manual woik ( ìrau-
maìít ICD ).
Irrìtaot keactìoo IC0 Eaily, subclinical dei-
matitis on hands of individuals exposed to wet
woik. Usually duiing fiist months of tiaining of
haii diesseis oi of metal woikeis, smaiting and
buining sensations.
CIINICAI MANIF£SIAII0N
Symptoms Stinging, smaiting, buining, anJ
itching; pain as fissuies develop.
Skìo Fìodìo¿s Diyness chapping eiythe-
ma (Fig. 2-3) hypeikeiatosis and scaling
fissuies and ciusting (Fig. 2-4). Shaip maigin-
ation gives way to ill-defined boideis, licheni-
fication. In írríìanì reatìíon ICD also vesicles,
pustules, and eiosions.
DIstrIhutIvn Usually on hands (Figs. 2-3 and
2-4). In tumu|aìí·e ICD usually staiting at
fingei webspaces, spieading to sides and doi-
sal suiface of hands and then to palms. In
housewives often staiting on fingeitips ( ¡u|¡í-
ìís ) (Fig. 2-3). Raiely in othei locations exposed
to iiiitants and/oi tiauma, e.g., in violinists on
mandible oi neck, oi on exposed sites as in aír-
|orne ICD (see below).
DurutIvn Chionic, months to yeais.
Coostìtutìooa| Symptoms
None, except when infection occuis. Chionic
ICD (e.g., hand deimatitis; see below) can
become a seveie occupational and emotional
pioblem.
IA80kAI0k¥ £XAMINAII0N
hìstopatho|o¿y In acute ICD, epideimal cell
neciosis, neutiophils, vesiculation, and necio-
sis. In chionic ICD, acanthosis, hypeikeiatosis,
lymphocytic infiltiate.
Fatch Iests These aie negative in ICD unless
alleigic contact deimatitis is also piesent (see
below).
SF£CIAI F0kMS 0F IC0
haod 0ermatìtìs
Most cases of chionic ICD occui on the hands
and aie occupational. Often sensitization to
alleigens (such as nickel oi chiomate salts)
occuis, and then ACD (acute and/oi chionic)
is supeiimposed on ICD. A typical example is
hand deimatitis in constiuction and cement
woikeis. Cement is alkaline and coiiosive, lead-
ing to chionic ICD; chiomates in cement sensi-
tize and lead to ACD (see Fig. 2-6). In such cases
the eiuption may spiead beyond the hands and
may even geneialize.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 23
FICük£ 2-1 Acute ìrrìtaot cootact dermatìtìs Io||owìo¿ app|ìcatìoo oI a cream cootaìoìo¿ oooy|va-
oì||amìd aod oìcotìoìc acìd-butoxyethy|ester prescrìbed Ior |ower back paìo I|e 'º||e+|, p+||e|u¨
|ud|c+|eº +u ou|º|de jo|. I|e e|up||ou |º c|+|+c|e||/ed |, + r+ºº|.e e|,||er+ W||| .eº|cu|+||ou +ud |||º|e|
|o|r+||ou +ud |º cou||ued |o ||e º||eº e\poºed |o ||e |o\|c +çeu|.
FICük£ 2-2 Acute ìrrìtaot cootact dermatìtìs oo the haod due to ao ìodustrìa| so|veot I|e|e |º
r+ºº|.e |||º|e||uç ou ||e p+|r.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 24
Aìrboroe IC0
Chaiacteiistically face, neck, anteiioi chest, and
aims aie involved. Most fiequent causes aie
iiiitating dust and volatile chemicals (ammo-
nia, solvents, foimaldehyde, epoxy iesins, ce-
ment, fibeiglass, sawdust fiom toxic woods).
This has to be distinguished fiom photoalleigic
contact deimatitis (see Section 10).
Fustu|ar aod AcoeìIorm IC0
ICD may taiget follicles and become pustulai
and papulopustulai. It may iesult fiom metals,
mineial oils, gieases, cutting fluids, naphtha-
lenes.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Diagnosis is by histoiy and clinical examination
(lesions, pattein, site). Most impoitant diffei-
ential diagnosis is ACD (see Table 2-3, p. 32).
On palms and soles: palmoplantai psoiiasis;
in exposed sites: photoalleigic contact deimatitis.
C0ükS£ AN0 Fk0CN0SIS
Healing usually occuis within 2 weeks of ie-
moval of noxious stimuli; in moie chionic cases,
6 weeks oi longei may be iequiied. In the setting
of occupational ICD, only one-thiid of indi-
viduals have complete iemission and two-thiids
may iequiie allocation to anothei job; atopic
individuals have a woise piognosis. In cases of
chionic subciitical levels of iiiitant, some woik-
eis develop toleiance, oi °haidening."
MANAC£M£NI
Freveotìoo
· Avoid iiiitant oi caustic chemical(s) by weai-
ing piotective clothing (i.e., goggles, shields,
gloves).
· If contact does occui, wash with watei oi
weak neutializing solution.
· Baiiiei cieams.
· In occupational ICD that peisists in spite of
adheience to the above measuies, change of
job may be necessaiy.
Ik£AIM£NI
Acute Identify and iemove the etiologic agent.
Wet diessings with gauze soaked in Buiow`s so-
lution, changed eveiy 2-3 h. Laigei vesicles may
be diained, but tops should noì be iemoved.
Topical class I glucocoiticoid piepaiations. In
seveie cases, systemic glucocoiticoids may be
indicated. Piednisone: 2-week couise, 60 mg
initially, tapeiing by steps of 10 mg.
Suhucute und ChrvnIc Identify and iemove
etiologic/pathogenic agent. Employ a potent
topical glucocoiticoid piepaiation, betametha-
sone dipiopionate oi clobetasol piopionate,
and piovide adequate lubiication. As healing
occuis, continue with lubiicating/piotective
cieams oi ointments. The topical calcineuiin in-
hibitois pimeciolimus and taciolimus aie usu-
ally not potent enough to suppiess the chionic
inflammation and its sequelae sufficiently.
In chionic ICD of hands a °haidening effect"
can be achieved in most cases with topical (soak
oi bath)-PUVA theiapy (see page 68).
FICük£ 2-3 £ar|y chrooìc ìrrìtaot cootact dermatìtìs ìo a housewìIe I||º |+º |eºu||ed ||or |epe+|ed
e\poºu|e |o ºo+pº +ud de|e|çeu|º. |o|e ç||º|eu|uç ||uçe|||pº (pu|p|||º).
S£CII0N 2 EC/E\A/|Ek\AI|I|' 25


FICük£ 2-4 . Chrooìc ìrrìtaot dermatìtìs wìth acute exacerbatìoo ìo a housewìIe I|e p+||eu| uºed
|u|peu||ue |o c|e+u |e| |+udº +||e| p+|u||uç. E|,||er+, ||ººu||uç, +ud ºc+||uç. ||||e|eu||+| d|+çuoº|º |º +||e|ç|c
cou|+c| de|r+||||º +ud p+|r+| pºo||+º|º. |+|c| |eº|º |o |u|peu||ue We|e ueç+||.e.  Irrìtaot cootact dermatìtìs |u
+ couº||uc||ou Wo||e| W|o Wo||º W||| cereu|. |o|e ||e |,pe||e|+|oºeº, ºc+||uç +ud ||ººu||uç. I|e|e |º +|ºo r|u|r+|
puº|u|+||ou.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 26
£FI0£MI0I0C¥
Fiequent. Accounts foi 7% of occupationally
ielated illnesses in the United States. How-
evei, theie aie data suggesting that the actual
indicence iate is 10 to 50 times gieatei than
iepoited in the U.S. Buieau of Laboi Statis-
tics data. Nonoccupational ACD is estimated
to be thiee times gieatei than occupational
ACD.
A¿e oI 0oset No influence on capacity foi
sensitization; howevei, alleigic contact deima-
titis is uncommon in young childien and in
individuals oldei than 70 yeais.
0ccupatìoo One of the most impoitant causes
of disability in industiy.
FAIh0C£N£SIS
ACD is a classic, delayed, cell-mediated hy-
peisensitivity ieaction. Exposuie to a stiong
sensitizei such as poison ivy iesin iesults in
sensitization in a week oi so, while exposuie
to a weak alleigen may take months to yeais
foi sensitization. The antigen is taken up by
Langeihans cells, which piocess the antigen
and migiate fiom the epideimis to the diaining
lymph nodes, wheie they piesent the piocessed
antigen in association with MHC class II mol-
ecules to T cells that then piolifeiate. Sensitized
T cells leave the lymph node, entei the blood
ciiculation, home to the skin, and, aftei being
piesented by Langeihans cells with the same
specific antigen, pioduce and mediate the ie-
lease by othei cells of a vaiiety of cytokines.
Thus, all the skin becomes hypeisensitive to
the contact alleigen and will ieact wheievei the
specific alleigen is piesented.
AII£kC£NS
Contact alleigens aie diveise and iange fiom
metal salts to antibiotics, dyes to plant pioducts.
AC| |º + º,º|er|c d|ºe+ºe de||ued |, |+p|eu·
ºpec|||c I ce||-red|+|ed |u||+rr+||ou.
0ue o| ||e roº| ||equeu|, .e\|uç, +ud coº||, º||u
p|o||erº.
Au ec/er+|ouº (p+pu|eº, .eº|c|eº, p|u||||c) de|·
r+||||º
|ue |o |ee\poºu|e |o + ºu|º|+uce |o W||c| ||e
|ud|.|du+| |º ºeuº|||/ed.
AII£kCIC C0NIACI 0£kMAIIIIS |C|·9 . o92.9

|C|·¹0 . |2+
Thus, alleigens aie found in jeweliy, peisonal
caie pioducts, topical medications, plants,
house iemedies, and chemicals the individual
may come in contact with at woik. The most
common alleigens in the United States aie listed
in Table 2-2.
CIINICAI MANIF£SIAII0N
The eiuption staits in a sensitized individual
48 h oi days aftei contact with the alleigen;
iepeated exposuies lead to a ciescendo ieac-
tion, i.e., the eiuption woisens. Site of the
eiuption is confined to site of exposuie. With
phytoalleigic (poison ivy), exposuie sites may
not be appaient to the patient. Haptens can
be blotted on to face oi penis without diiect
contact.
Symptoms Subjective symptoms aie intense
piuiitus; in seveie ieactions also stinging and
pain.
Coostìtutìooa| Symptoms °Acute illness" syn-
diome, including fevei, but only in seveie allei-
gic contact deimatitis (e.g., poison ivy).
Skìo Iesìoos The appeaiance of ACD depends
on seveiity, location, and duiation.
Iype Acute Well-demaicated eiythema
and edema on which aie supeiimposed closely
spaced, nonumbilicated vesicles, and/oi papules
(Fig. 2-5); in seveie ieactions, bullae, confluent
eiosions exuding seium, and ciusts. The same
ieaction can occui aftei seveial weeks at sites
not exposed.
Suhucute Plaques of mild eiythema showing
small, diy scales, sometimes associated with
small, ied, pointed oi iounded (Figs. 2-6, 2-7),
fiim papules.
ChrvnIc Plaques of lichenification (thicken-
ing of the epideimis with deepening of the skin
lines in paiallel oi ihomboidal pattein), scaling
with satellite, small, fiim, iounded oi flat-
topped papules, excoiiations, eiythema, and
pigmentation.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 2T
FICük£ 2-5 Acute a||er¿ìc cootact dermatìtìs oo the |ìps due to |ìpstìck I|e p+||eu| W+º |,pe|ºeuº|·
||.e |o eoº|u. |o|e |||ç|| e|,||er+, r|c|o.eº|cu|+||ou. A| c|oºe |uºpec||ou + p+pu|+| corpoueu| c+u |e d|ºce|ued.
A| |||º º|+çe ||e|e |º º|||| º|+|p r+|ç|u+||ou.
FICük£ 2-6 A||er¿ìc cootact dermatìtìs oI haods:
chromates Cou||ueu| p+pu|eº, .eº|c|eº, e|oº|ouº +ud
c|uº|º ou ||e do|ºur o| ||e |e|| |+ud |u + couº||uc||ou
Wo||e| W|o W+º +||e|ç|c |o c||or+|eº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 28
IA8I£ 2-2 Iop Ien Contact A||er¿ens (North American Contact 0ermatitis Croup)
and 0ther Common Contact A||er¿ens
*
A||ergeo Pr|oc|pa| So0rces oI 0ootact
||c|e| ºu||+|e \e|+|º, re|+|º |u c|o|||uç, jeWe||,, c+|+|,/|uç +çeu|º
|eor,c|u ºu||+|e uºu+||, cou|+|ued |u c|e+rº, o|u|reu|º
B+|º+r o| |e|u Iop|c+| red|c+||ouº
||+ç|+uce r|\ ||+ç|+uceº, coºre||cº
I||re|oº+| Au||ºep||cº
'od|ur ço|d |||oºu||+|e \ed|c+||ou
|o|r+|de|,de ||º|u|ec|+u|, cu||uç +çeu|º, p|+º||cº
0u+|e|u|ur·¹5 ||º|u|ec|+u|
B+c|||+c|u 0|u|reu|º, poWde|
Co|+|| c||o||de Cereu|, ç+|.+u|/+||ou, |uduº|||+| o||º, coo||uç +çeu|º, e,eº|+deº
\e||,|d|||oroç|u|+|ou|||||e, p|euo\,|e||+uo| ||eºe|.+||.eº, coºre||cº
C+||+ r|\ ku||e|, |+|e\
|+|+·p|eu,|eud|+r|ue B|+c| o| d+|| d,eº o| |e\|||eº, p||u|e|'º |u|
I||u|+r ku||e|
|+|+|,d|o\,|eu/o|c +c|d eº|e| Couºe|.|uç +çeu| |u |oodº|u||º
||op,|eue ç|,co| ||eºe|.+||.eº, coºre||cº
||oc+|ue, |eu/oc+|ue |oc+| +ueº||e||cº
'u||ou+r|deº \ed|c+||ou
Iu|peu||ue 'o|.eu|º, º|oe po||º|, p||u|e|'º |u|
\e|cu|, º+||º ||º|u|ec|+u|, |rp|eçu+||ou
C||or+|eº Cereu|, +u||o\|d+u|º, |uduº|||+| o||º, r+|c|eº, |e+||e|
|+|+|eueº B|oc|deº, p|eºe|.+||.eº
C|uu+r|c +|de|,de ||+ç|+uce, pe||ure
|eu|+dec,|c+|ec|o|º ||+u|º, e.ç., po|ºou |.,
* 0.e| !100 c|er|c+|º |+.e |eeu |epo||ed |o c+uºe AC|.
Arrao¿emeot Initially, confined to aiea of
contact with alleigen ¦e.g., eailobe (eaiiings),
doisum of foot (shoes), wiist (watch oi watch-
band), collai-like (necklace), lips (lipstick)].
Often lineai, with aitificial patteins, an °outside
job." Plant contact often iesults in lineai lesions
(e.g., R|us deimatitis). Initially confined to site
of contact, latei spieading beyond.
0ìstrìbutìoo Ertent Isolated, localized to
one iegion (e.g., shoe deimatitis), oi geneial-
ized (e.g., plant deimatitis).
Puttern Random oi on exposed aieas (as in
aiiboine ACD).
C0ükS£
£vo|utìoo oI AC0 The duiation of ACD vaiies
among individuals, iesolving in some in 1-2
weeks. ACD continues to get woise as long as
alleigen continues to come into contact with
the skin.
Acute Eiythema papules vesicles
eiosions ciusts scaling.
Noìe. In the acute foims of contact deimati-
tis, papules occui only in ACD, not in ICD.
ChrvnIc Papules scaling licheni-
fication excoiiations. Chionic inflam-
mation with thickening, fissuiing, scaling,
and ciusting iesults.
Noìe. Contact deimatitis is always con-
fined to the site of exposuie to the alleigen.
Maigination is oiiginally shaip in ACD; how-
evei, it spieads in the peiipheiy beyond the
actual site of exposuie. If stiong sensitiza-
tion has occuiied, spieading to othei paits
of the body and geneialization occui. The
main diffeiences between toxic iiiitant and
alleigic contact deimatitis aie summaiized in
Table 2-3.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 29
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Acute Piototype of
spongiotic deimatitis. Inflammation with in-
tiaepideimal inteicellulai edema ( s¡ongíosís ),
lymphocytes and eosinophils in the epideimis,
and monocyte and histiocyte infiltiation in the
deimis.
ChrvnIc In chionic ACD theie aie also spon-
giosis plus acanthosis, elongation of iete iidges,
and elongation and bioadening of papillae; hy-
peikeiatosis; and a lymphocytic infiltiate.
Fatch Iests In ACD sensitization is piesent
on eveiy pait of the skin; theiefoie, application
of the alleigen to any aiea of noimal skin pio-
vokes an eczematous ieaction. A positive patch
test shows eiythema and papules, as well as
possibly vesicles confined to the test site. Patch
tests should be delayed until the deimatitis has
subsided foi at least 2 weeks and should be pei-
foimed on a pieviously uninvolved site.
(See °Clinical Tests," Intioduction.)
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
By histoiy and clinical findings, including eval-
uation of site and distiibution. Histopathology
Ie|red c||-·¸·: ¤|,|¤!-·¤c|·|·º (A||)
0ccu|º |u ºeuº|||/ed |ud|.|du+|º +||e| e\poºu|e
|o + W|de .+||e|, o| p|+u| +||e|çeuº
C|+|+c|e||/ed |, +u +cu|e, .e|, p|u||||c, ec/er·
+|ouº de|r+||||º, o||eu |u + ||ue+| +||+uçereu|
|u ||e uu||ed '|+|eº, po|ºou |.,/o+| +|e |, |+|
||e roº| corrou p|+u|º |rp||c+|ed
|¤|- ||,|¤¤|¤|¤!-·¤c|·|·º |º + d|||e|eu| eu|||,,
|| |º + p|o|oºeuº|||.||, |e+c||ou occu|||uç |u +u,
|ud|.|du+| W||| + p|o|oºeuº|||/|uç p|+u|-de||.ed
c|er|c+| ou ||e º||u +ud ºu|ºequeu| ºuu e\po·
ºu|e (ºee 'ec||ou ¹0)
AII£kCIC C0NIACI 0£kMAIIIIS
0ü£ I0 FIANIS
may be helpful; veiification of offending agent
(alleigen) by patch test. Exclude ICD (Table
2-3), atopic deimatitis, seboiiheic deimatitis
(face), psoiiasis (palms and soles), epideimal
deimatophytosis (KOH), fixed diug eiuption,
eiysipelas phytophotodeimatitis.
SF£CIAI F0kMS 0F AC0
FICük£ 2-T A||er¿ìc cootact dermatìtìs due to oìcke|, subacute |o|e + r|\ o| p+pu|+|, .eº|cu|+|, +ud
c|uº|ed |eº|ouº +ud |oºº o| º|+|p r+|ç|u+||ou. I|e p+||eu| W+º + |e|||ed W+|c|r+|e| W|o uºed + re|+| c|+ºp ou
||e do|ºur o| ||e |e|| |+ud W|||e |ep+|||uç W+|c|eº. ne W+º |uoWu |o |e +||e|ç|c |o u|c|e|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 30
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset Occuis in individuals of all ages.
Veiy young and veiy old aie less likely to be sen-
sitized to plants. Sensitization is lifelong.
£tìo|o¿y Pentadecylcatechols, piesent in the
Anacaidiaceae plant family, aie the most com-
mon sensitizeis in the United States. They
cioss-ieact with othei phenolic compounds
such as iesoicinol, hexyliesoicinol, and hy-
dioxyquinones.
F|aots AnucurdIuceue FumI|y Poison ivy
(ToxítoJenJron raJítans ) and poison oak ( T.
querí[o|íum, T. Jí·ersí|o|um ) . Also poison
sumac ( T. ·erníx ). Plants ielated to poison
ivy gioup: Biazilian peppei, cashew nut tiee,
ginkgo tiee, Indian maikei nut tiee, lacquei
tiee, mango tiee, iengas tiee.
Ceo¿raphy Poison ivy occuis thioughout the
United States (except extieme southwest) and
southein Canada; poison oak on the west coast.
Poison sumac and poison dogwood giow only
in woody, swampy aieas.
£xposure Telephone and electiical woikeis
woiking outdoois. Leaves, stems, seeds, floweis,
beiiies, and ioots contain milky sap that tuins
to a black iesin on exposuie to aii. Cashew oil:
unioasted cashew nuts (heat destioys hapten);
cashew oil in wood (Haitian voodoo dolls,
swizzle sticks), iesins, piintei`s ink. Mango
iind. Maiking nut tiee of India: laundiy maikei
(dhobi itch). Fuinituie lacquei fiom Japanese
lacquei tiee.
Seasoo APD usually occuis in the spiing,
summei, and fall; can occui yeai-iound if ex-
posed to stems oi ioots. In southwest of the
United States, occuis yeai-iound.
FAIh0C£N£SIS
All ToxítoJenJron plants contain identical al-
leigens. Hapten is piesent in milky sap in
leaves, stems, seeds, floweis, beiiies, and ioots.
The oleoiesins aie iefeiied to as urus|ío| . The
haptens aie the pentadecylcatechols (1, 2-
hydioxybenzenes with a 15-caibon side chain
in position thiee). Washing with soap and watei
iemoves oleoiesins.
Moie than 70% of individuals can be sensi-
tized to ToxítoJenJron haptens. Daik-skinned
individuals aie less susceptible to APD. Aftei
fiist exposuie (sensitization) deimatitis oc-
cuis 7-12 days latei. In a pieviously sensitized
peison (may be many decades befoie), dei-
matitis occuis (especially on face oi genitalia)
in 12 h aftei ieexposuie. Diffeience in clini-
cal couise vaiies with individual ieactivity,
inoculum of hapten on skin, and iegional
vaiiation.
Noìe. Blistei fluid does not contain hapten
and cannot spiead the deimatitis; exposuie to
smoke fiom the buining plant is haimless, but
deimatitis can occui fiom paiticulate mattei in
the smoke.
CIINICAI MANIF£SIAII0N
£xposure PvIsvn 1vy/Ou| DermutItIs Diiect
plant exposuie: plant biushes against exposed
skin giving iise to lineai lesions (Fig. 2-8); iesin
usually is not able to penetiate the thick stia-
tum coineum of palms/soles. Clothing: weaiing
clothing pieviously contaminated with iesin
can ieexpose the skin.
Fvvd CvntuInIng UrushIv| Eating unpeeled
mango oi unioasted cashew nuts can expose
lips to oleoiesin. Mucous membianes uncom-
monly expeiience APD, but ingestion of uiu-
shiol can pioduce alleigic contact deimatitis of
the anus and peiineum.
Skìo Symptoms Piuiitus mild to seveie. Often
sensed befoie any detectable skin changes. Pain
in some cases. Secondaiy infection associated
with local tendeiness.
Coostìtutìooa| Symptoms Sleep depiivation
due to piuiitus.
Skìo Iesìoos Initially, well-demaicated patch-
es of eiythema, chaiacteiistic lineai lesions (Fig.
2-8); iapidly evolve into papules and edematous
plaques; may be seveie especially on face and/oi
genitals, iesembling cellulitis (Figs. 2-9, 35-16)
Miciovesiculation may evolve to vesicles and/
oi bullae (Figs. 2-8 and 2-10). Eiosions, ciusts.
With iesolution, eiythematous plaques  scale,
eiosion, ciusting. Postinflammatoiy hypei-
pigmentation common in daikei skinned indi-
viduals.
DIstrIhutIvn Most commonly on exposed ex-
tiemities, wheie contact with the plant occuis;
blotting can tiansfei to any exposed site; palms/
soles aie usually spaied; howevei, lateial fingeis
can be involved.
C|vthIng-Prvtected SItes Oleoiesin can pen-
etiate damp clothing onto coveied skin.
Nvnerpvsed SItes °Id"-like ieaction oi some
systemic absoiption can be associated with
disseminated uiticaiial, eiythema multifoime-
like, oi scailatinifoim lesions away fiom sites
of exposuie in some individuals with well-
established APD.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 31
FICük£ 2-8 A||er¿ìc phytodermatìtìs
oI |e¿: poìsoo ìvy ||ue+| .eº|cu|+| |eº|ouº
W||| e|,||er+ +ud eder+ ou ||e c+|| +| º||eº
o| d||ec| cou|+c| o| ||e º||u 5 d+,º +||e| e\po·
ºu|e W||| ||e po|ºou |., |e+|.
FICük£ 2-9 A||er¿ìc phytoderma-
tìtìs oI Iace: poìsoo ìvy \e|, p|u||||c
e|,||er+, eder+, r|c|o.eº|cu|+||ou o| ||e
c|ee|º +ud pe||o||||+| +|e+ |u + p|e.|ouº|,
ºeuº|||/ed 1·,e+|·o|d |o,, occu|||uç ! d+,º
+||e| e\poºu|e.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y See ACD, above.
Fatch Iests wìth Feotadecy|catecho|s Con-
tiaindicated. Can sensitize the individual to
hapten.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 32
0IACN0SIS
By histoiy and clinical findings.
0IFF£k£NIIAI 0IACN0SIS
ACD to othei alleigens, phytophotodeimatitis
(see Section 10), soft-tissue infection (celluli-
tis, eiysipelas), atopic deimatitis, inflammatoiy
deimatophytosis, eaily heipes zostei, fixed diug
eiuption.
IA8I£ 2-3 0ifferences Between |rritant and A||er¿ic Contact 0ermatitis*
|rr|taot 00 A||erg|c 00
',rp|orº Acu|e Stìo¿ìo¿, smartìo¿ ìtchìo¿ Itchìo¿ paìo
C||ou|c ||c||uç/p+|u ||c||uç/p+|u
|eº|ouº Acu|e E|,||er+ .eº|c|eº E|,||er+ papu|es
e|oº|ouº c|uº|º ºc+||uç .eº|c|eº e|oº|ouº c|uº| ºc+||uç
C||ou|c |+pu|eº, p|+queº, ||ººu|eº, |+pu|eº, p|+queº, ºc+||uç,
ºc+||uç, c|uº|º c|uº|º
\+|ç|u+||ou Acu|e Sharp, strìct|y cooIìoed '|+|p, cou||ued |o º||e o| e\poºu|e
+ud º||e to sìte oI exposure but spreadìo¿ ìo the perìphery,
usua||y tìoy papu|es, may become
¿eoera|ìted
C||ou|c |||·de||ued |||·de||ued, spreads
E.o|u||ou Acu|e kapìd (|eW |ou|º +||e| e\poºu|e) Not so rapìd (¹2-12 | +||e| e\poºu|e)
C||ou|c \ou||º |o ,e+|º o| |epe+|ed e\poºu|e \ou||º o| |ouçe|, e\+ce||+||ou +||e|
e.e|, |ee\poºu|e
C+uº+||.e 0epeodeot oo cooceotratìoo oI a¿eot ke|atìve|y ìodepeodeot oI amouot
+çeu|º aod state oI skìo barrìer, occurs oo|y app|ìed, usua||y very |ow
above thresho|d |eve| cooceotratìoos suIIìcìeot but depeods
oo de¿ree oI seosìtìtatìoo
|uc|deuce May occur ìo practìca||y everyooe 0ccurs oo|y ìo the seosìtìted
*
||||e|euceº +|e p||u|ed |u |o|d.
Cou|+c| W||| +|||o|ue +||e|çeuº |u e\poºed |od,
º||eº, uo|+||, ||e |+ce (||ç. 2·¹¹), +|ºo |uc|ud|uç
e,e||dº, '\¨ o| ||e uec|, +|rº, +ud |eçº.
|u cou||+º| |o +|||o|ue |C|, p+pu|+| ||or ||e
|eç|uu|uç, e\||ere|, ||c|,.
||o|ouçed |epe||||.e e\poºu|e |e+dº |o d|,,
||c|eu|||ed AC| W||| e|oº|ouº +ud c|uº||uç (||ç.
2·¹¹).
|ue |o p|+u| +||e|çeuº, eºpec|+||, ||or cor·
poº||+e, u+|u|+| |eº|uº, Woodº, eººeu||+| o||º
.o|+||/|uç ||or +|or+ ||e|+p,.
AIk80kN£ AC0
MANAC£M£NI 0F AC0
Iermìoatìoo oI £xposure Identify and iemove
the etiologic agent.
A||e| º,º|er|c e\poºu|e |o +u +||e|çeu |o W||c|
||e |ud|.|du+| |+d p||o| AC|.
A de|+,ed I ce||-red|+|ed |e+c||ou.
E\+rp|eº. AC| |o e||,|eued|+r|ue ºu|ºe·
queu| |e+c||ou |o +r|uop|,|||ue (W||c| cou|+|uº
e||,|eue d|+r|ue), po|ºou |., de|r+||||º
ºu|ºequeu| |e+c||ou |o |uçeº||ou o| c+º|eW
uu|º, +|ºo +u||||o||cº, ºu||ou+r|deº, p|op,|eue
ç|,co|, re|+| |ouº, ºo|||c +c|d, ||+ç|+uceº.
S¥SI£MIC AC0 (SAC0)
S£CII0N 2 EC/E\A/|Ek\AI|I|' 33
Iopìca| Iherapy Topical glucocoiticoid oint-
ments/gels (classes I to III) aie effective foi
eaily nonbullous lesions. Laigei vesicles may be
diained, but tops should not be iemoved. Wet
diessings with cloths soaked in Buiow solu-
tion changed eveiy 2-3 h. Since tieatment with
glucocoiticoids is usually shoit-teim in ACD,
theie is usually no dangei of glucocoiticoid side
effects. An exception is aiiboine ACD, which
may iequiie systemic tieatment. The topical
calcineuiin inhibitois pimeciolimus and tacio-
limus aie effective in ACD but to a lessei degiee
than glucocoiticoids.
Systemìc Iherapy Glucocoiticoids aie indi-
cated if seveie (i.e., if patient cannot peifoim
usual daily functions, cannot sleep). Piednisone
beginning at 70 mg (adults), tapeiing by 5-10
mg/d ovei a 1- to 2-week peiiod.
In aiiboine ACD wheie complete avoidance
of alleigen may be impossible, immunosup-
piession with oial cyclospoiine may become
necessaiy.
FICük£ 2-10 Acute a||er¿ìc phytodermatìtìs, bu||ous I||º e|up||ou occu||ed |u + p+||eu| W|o |+d W+||ed |+|e·
|oo| |||ouç| + |o|eº|. || |+|e| ºp|e+d +º + p+pu|+| e|up||ou |o ||e |eº| o| ||e |od,. '|r||+| |eº|ouº We|e p|eºeu| ou ||e o||e|
|oo| +ud |oWe| |eç. ||||e|eu||+| d|+çuoº|º |uc|uded +cu|e |u||ouº cou|+c| de|r+||||º |o c+|e|p|||+|º. ||,|op|o|ode|r+||||º
W+º e\c|uded |ec+uºe +| ||e ||re o| e\poºu|e ||e|e W+º + |e+.||, c|ouded º|, +ud + p+pu|+| e|up||ou occu||ed |+|e| ou.
C+|e|p|||+| de|r+||||º W+º e\c|uded |ec+uºe o| ||e ru|||p||c||, o| ||e |eº|ouº +ud |ec+uºe upou p+|c| |eº||uç ||e p+||eu| W+º
poº|||.e |o |o\|codeud|ou |+p|euº. |o|e, p+|c| |eº||uç |o u|uº||o| |º uo |ouçe| doue |o +.o|d ºeuº|||/+||ou o| p+||eu|º.
FICük£ 2-11 Aìrboroe a||er¿ìc cootact dermatìtìs
oo the Iace E\||ere|, ||c|,, cou||ueu| p+pu|+|, e|oº|.e,
+ud c|uº|ed/ºc+|, |eº|ouº W||| ||c|eu|||c+||ou ou ||e |o|e|e+d,
|o||oW|uç e\poºu|e |o p|ueWood duº|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 34
£FI0£MI0I0C¥
A¿e oI 0oset Fiist 2 months of life and by the
fiist yeais in 60% of patients. 30% aie seen foi
the fiist time by age 5, and only 10% develop
AD between 6 and 20 yeais of age. Raiely AD
has an adult onset.
Ceoder Slightly moie common in males than
females.
Freva|eoce Between 7 and 15% iepoited in
population studies in Scandinavia and Gei-
many.
Ceoetìc Aspects The inheiitance pattein has
not been asceitained. Howevei, in one seiies,
60% of adults with AD had childien with AD.
The pievalence in childien was highei (81%)
when both paients had AD.
£|ìcìtìo¿ Factors 1nhu|unts Specific aeioal-
leigens, especially dust mites and pollens, have
been shown to cause exaceibations of AD.
MIcrvhIu| Agents Exotoxins of Sìa¡|y|otottus
aureus may act as supeiantigens and stimulate
activation of T cells and maciophages.
Autvu||ergens Seia of patients with AD
contain IgE antibodies diiected at human pio-
teins. The ielease of these autoalleigens fiom
damaged tissue could tiiggei IgE oi T cell
iesponses, suggesting maintenance of alleigic
inflammation by endogenous antigens.
Fvvds Su|seì of infants and childien have
flaies of AD with eggs, milk, peanuts, soybeans,
fish, and wheat.
0ther £xacerbatìo¿ Factors
S|In BurrIer DIsruptIvn: deciease of baiiiei
function associated with impaiied filag-
giin pioduction, ieduced ceiamide levels,
and incieased tiansepideimal watei loss
Au +cu|e, ºu|+cu|e, o| c||ou|c |e|+pº|uç º||u
d|ºo|de|
uºu+||, |eç|uº |u |u|+uc,
||e.+|+uce pe+| o| ¹5-20° |u e+||, c|||d|ood
C|+|+c|e||/ed p||uc|p+||, |, d|, º||u +ud p|u|||uº,
couºequeu| |u|||uç |e+dº |o |uc|+ºed |u||+rr+·
||ou +ud ||c|eu|||c+||ou +ud |o |u|||e| ||c||uç +ud
ºc|+|c||uç. ·|:|·º:·c|:| :,:|-
||+çuoº|º |º |+ºed ou c||u|c+| ||ud|uçº
0||eu +ººoc|+|ed W||| + pe|ºou+| o| |+r||, ||º|o|,
o| A|, +||e|ç|c |||u|||º, +ud +º||r+, !5° o| |u|+u|º
W||| A| de.e|op +º||r+ |+|e| |u |||e
Aººoc|+|ed W||| º||u |+|||e| d,º|uuc||ou, |çE |e+c·
||.||,
Ceue||c |+º|º |u||ueuced |, eu.||oureu|+| |+c·
|o|º, +||e|+||ouº |u |rruuo|oç|c |eºpouºeº |u
I ce||º, +u||çeu p|oceºº|uç, |u||+rr+|o|, c,|o||ue
|e|e+ºe, +||e|çeu ºeuº|||.||,, |u|ec||ou
',¤¤¤,¤º. |çE de|r+||||º, 'ec/er+,¨ +|op|c ec·
/er+.
AI0FIC 0£kMAIIIIS |C|·9 . o9¹.3

|C|·¹0 . |20
by fiequent bathing and hand washing;
dehydiation is an impoitant exaceibating
factoi.
1n]ectIvns: S. aureus is almost always piesent
in seveie cases; gioup A stieptococcus;
iaiely fungus (deimatophytosis, candidia-
sis).
Seusvn: in tempeiate climates, AD usually
impioves in summei, flaies in wintei.
C|vthIng: piuiitus flaies a[ìer taking off
clothing. Wool is an impoitant tiiggei;
wool clothing oi blankets diiectly in con-
tact with skin (also wool clothing of pai-
ents, fui of pets, caipets).
EmvtIvnu| Stress: iesults fiom the disease oi
is itself an exaceibating factoi in flaies of
the disease.
FAIh0C£N£SIS
Complex inteiaction of skin baiiiei, genetic,
enviionmental, phaimacologic, and immuno-
logic factois. Type I (IgE-mediated) hypeisen-
sitivity ieaction occuiiing as a iesult of the
ielease of vasoactive substances fiom both mast
cells and basophils that have been sensitized
by the inteiaction of the antigen with IgE (ie-
aginic oi skin-sensitizing antibody). The iole
of IgE in AD is still not fully claiified, but epi-
deimal Langeihans cells possess high-affinity
IgE ieceptois thiough which an eczema-like
ieaction can be mediated. T
H
2 and T
H
1 both
contiibute to skin inflammation in AD. Actute
T cell infiltiation in AD is associated with a
piedominance of inteileukin (IL) 4 and IL-13
expiession, and chionic inflammation in AD
S£CII0N 2 EC/E\A/|Ek\AI|I|' 35
FICük£ 2-12 Atopìc dermatìtìs: ìoIaotì|e |u||, |+ce, cou||ueu| e|,||er+, p+pu|eº, r|c|o.eº|cu|+||ou, ºc+|·
|uç, +ud c|uº||uç
FICük£ 2-13 Atopìc dermatìtìs: ìoIaotì|e-type '||u o| |o|e|e+d |º d|,, c|+c|ed +ud ºc+|,. |u +dd|||ou, ||e|e
+|e oo/|uç e|oº|ouº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 36
with incieased IL-5, gianulocyte-maciophage
colony-stimulating factoi (GM-CSF), IL-12,
and inteifeion (IFN) . Thus, skin inflamma-
tion in AD shows a biphasic pattein of T cell
activation.
CIINICAI MANIF£SIAII0N
Skìo Symptoms Patients have diy skin. Piuii-
tus is the sine qua non of atopic deimatitis-
°eczema is the itch that iashes." The constant
sciatching leads to a vicious cycle of itch
sciatch iash itch sciatch.
0ther Symptoms oI Atopy Alleigic ihinitis,
chaiacteiized by sneezing, ihinoiihea, obstiuc-
tion of nasal passages, conjuctival and phaiyn-
geal itching, and laciimation; seasonal when
associated with pollen.
Skìo Iesìoos Acute Pooily defined eiy-
thematous patches, papules, and plaques with
oi without scale. Edema with widespiead in-
volvement; skin appeais °puffy" and edematous
(Fig. 2-12). Eiosions: moist, ciusted. Lineai oi
punctate, iesulting fiom sciatching. Secondaiily
infected sites: S. aureus. Oozing eiosions (Figs.
2-12 and 2-13) and/oi pustules (usually follicu-
lai). Skin may be extiemely diy and ciacked and
scaly (Fig. 2-13).
ChrvnIc Lichenification (thickening of the
skin with accentuation of skin maikings): iesults
fiom iepeated iubbing oi sciatching (Figs. 2-14
and 2-15); folliculai lichenification (especially
in biown and black peisons) (Fig. 2-16). Fis-
suies: painful, especially in flexuies (Fig. 2-15),
on palms, fingeis, and soles. Alopecia: lateial
one-thiid of the eyebiows as a iesult of iubbing.
Peiioibital pigmentation: also as a iesult of
compulsive iubbing . Chaiacteiistic infiaoi-
bital fold below eyelids (Dennie-Moigan sign).
DIstrIhutIvn Piedilection foi the flex-
uies, fiont and sides of the neck, eyelids,
foiehead, face, wiists, and doisa of the feet
and hands (Image 2-1). Geneialized in seveie
disease (Fig. 2-17).
Specìa| Features ke|ated to A¿e
1n]untI|e AD The lesions piesent as ied skin,
tiny vesicles on °puffy" suiface. Scaling, exuda-
tion with wet ciusts and ciacks (fissuies) (Figs.
2-12 to 2-14). Skin lesions seem to be a ieaction
to itching and iubbing.
ChI|dhvvd-type AD The lesions aie papulai,
lichenified plaques, eiosions, ciusts, especially
on the antecubital and popliteal fossae (Figs.
2-15, to 2-17), the neck and face; may be gen-
eialized.
Adu|t-type AD Theie is a similai distiibution,
mostly flexuial but also face and neck, with
lichenification and exoiiations being the most
conspicuous symptoms (Figs. 2-18, 2-19). May
be geneialized.
Specìa| Features ke|ated to £thoìcìty
In blacks but also daik-biown skin, so-called
folliculai eczema is common; chaiacteiized
by disciete folliculai papules (Figs. 2-16, 2-19,
2-20) involving haii follicles of the involved
site.
Assocìated Fìodìo¿s
°White" deimatogiaphism is a special and
unique featuie of involved skin: stioking will
not lead to iedness as in noimal skin but to
blanching ; delayed blanch to cholineigic
agents. It|ì|yosís ·u|garís and |eraìosís ¡í|arís
(see page 75) occui in 10% of patients. Veinal
conjuctivitis with papillaiy hypeitiophy oi cob-
blestoning of uppei eyelid conjuctiva. Atopic
keiatoconjunctivitis is disabling, may iesult in
coineal scaiiing. Keiatoconus iaie. Cataiacts in
a small peicentage.
0IACN0SIS
Histoiy in infancy, clinical findings (typical
distiibution sites, moiphology of lesions, white
deimatogiaphism).
0IFF£k£NIIAI 0IACN0SIS
Seboiiheic deimatitis, ICD, ACD, psoiiasis,
nummulai eczema, deimatophytosis, eaily
stages of mycosis fungoides. Raiely, aciodei-
matitis enteiopathica, glucagonoma syndiome,
histidinemia, phenylketonuiia; also, some im-
munologic disoideis including Wiskott-Aldiich
syndiome, X-linked agammaglobulinemia, hy-
pei-IgE syndiome, and selective IgA deficiency;
Langeihans cell histiocytosis, Letteiei-Siwe
type.
IA80kAI0k¥ £XAMINAII0NS
8acterìa| Cu|ture Colonization with S. aureus
veiy common in the naies and in the involved
skin; almost 90% of patients with seveie AD aie
secondaiily colonized/infected. Look out foi
methicillin-iesistant S. aureus (MRSA).
vìra| Cu|ture Rule out heipes simplex viius
(HSV) infection in ciusted lesions (eczema
heipeticum; see Section 27).
S£CII0N 2 EC/E\A/|Ek\AI|I|' 3T
FICük£ 2-14 Chì|dhood atopìc dermatìtìs A |,p|c+| |oc+||/+||ou o| +|op|c de|r+||||º |u c|||d|eu |º ||e |eç|ou
+|ouud ||e rou||. |u |||º c|||d ||e|e |º ||c|eu|||c+||ou +ud ||ººu||uç +ud c|uº||uç.
FICük£ 2-15 Chì|dhood atopìc dermatìtìs 0ue o| ||e |+||r+||º o| +|op|c de|r+||||º |º ||c|eu|||c+||ou |u ||e
||e\u|+| |eç|ouº +º º|oWu |u |||º p|c|u|e. |o|e ||e |||c|eu|uç o| ||e º||u W||| e\+ççe|+|ed º||u ||ueº +ud e|oº|ouº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 38
8|ood Studìes Incieased IgE in seium, eosino-
philia. HSV antigen detection foi diagnosis of
acute HSV infection.
0ermatopatho|o¿y Vaiious degiees of acan-
thosis with iaie intiaepideimal inteicellulai
edema (spongiosis). The deimal infiltiate is
composed of lymphocytes, monocytes, and
mast cells with few oi no eosinophils.
SF£CIAI F0kMS 0F A0
Hund DermutItIs Aggiavated by wetting and
washing with deteigents, haish soaps, and Jís-
ín[etìanìs, leads to ICD in the atopic. Clini-
cally indistinguishable fiom °noimal" ICD
(see p. 22).
Er]v|IutIve DermutItIs (See SectIvn 8) Eiyth-
iodeima in patients with extensive skin
involvement. Geneialized iedness, scaling,
weeping, ciusting, lymphadenopathy, fevei, and
systemic toxicity.
C0MFIICAII0NS
Secondaiy infection with S. aureus and
heipes simplex viius (eczema heipeticum, see
Section 27). Raiely keiatoconus, cataiacts and
keiatoconjunctivitis with secondaiy heipetic
infection and coineal ulceis.
C0ükS£ AN0 Fk0CN0SIS
Untieated involved sites peisist foi months
oi yeais. Spontaneous, moie oi less complete
iemission duiing childhood occuis in >40%
with occasional, moie seveie iecuiiences dui-
ing adolescence. In many patients, the disease
peisists foi 15-20 yeais, but is less seveie. Fiom
30 to 50% of patients develop asthma and/oi
hay fevei. Adult-onset AD often iuns a seveie
couise. S. aureus infection leads to extensive
eiosions and ciusting, and heipes simplex in-
fection to eczema heipeticum, which may be
life-thieatening (see Section 27).
MANAC£M£NI
Education of the patient to avoid iubbing and
sciatching is most impoitant. Topical antipiu-
iitic (menthol/camphoi) lotions aie helpful in
contiolling the piuiitus but aie useless if emol-
lients aie not used and the patient continues to
sciatch and iub the plaques.
An alleigic woikup is
iaiely helpful in uncovei-
ing an alleigen; howevei,
in patients who aie hy-
peisensitive to house dust
mites, vaiious pollens,
and animal haii pioteins,
exposuie to the appio-
piiate alleigen may cause
flaies. Atopic deimatitis is
consideied by many to be
ielated, at least in pait, to
emotional stiess. It may
exaceibate with sweating.
Patients should be
wained of theii special
pioblems with heipes
simplex and the fiequency
of supeiimposed staphy-
lococcal infection, foi
which oial antibiotics aie
indicated. Antiviial diugs
foi heipes simplex aie in-
dicated if HSV infection
is suspected.
IMAC£ 2-1 Fredì|ectìoo sìtes oI atopìc dermatìtìs.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 39
Acute
1. Wet diessings and topical glucocoiticoids;
topical antibiotics (mupiiocin ointment)
when indicated.
2. Hydioxyzine, 10-100 mg foui times daily
foi piuiitus.
3. Oial antibiotics (dicloxacillin, eiythiomy-
cin) to eliminate S. aureus and tieat MRSA
accoiding to sensitivity as shown by cultuie.
Subacute aod Chrooìc
1. Hydiation (oilated baths oi baths with oat-
meal powdei) followed by application of
unscented emollients (e.g., hydiated petiola-
tum) foim the basic daily tieatment needed
to pievent xeiosis. Soap showeis aie peimis-
sible to wash the body folds, but soap should
seldom be used on the othei paits of the skin
suiface. 12% ammonium lactate oi 10% -
hydioxy acid lotion is veiy effective foi the
xeiosis seen in AD.
2. Topical anti-inflammatoiy agents such as
glucocoiticoids, hydioxyquinoline piep-
aiations, and tai aie the mainstays of
tieatment. Of these, glucocoiticoids aie the
most effective. Howevei, topical glucocoi-
ticoids may lead to skin atiophy if used
foi piolonged peiiods of time and if used
excessively will lead to suppiession of the
pituitaiy-adienal axis, osteopoiosis, giowth
ietaidation. Anothei pioblem is °glucocoi-
ticoidophobia." Patients oi theii paients aie
incieasingly awaie of glucocoiticoid side
effects and iefuse theii use, no mattei how
beneficial they may be.
3. New topical nonsteioidal anti-inflammatoiy
agents, the calcineuiin inhibitois taciolimus
and pimeciolimus, aie giadually ieplacing
glucocoiticoids in most patients. They po-
tently suppiess itching and inflammation
and do not lead to skin atiophy. They aie
usually not effective enough to suppiess
acute flaies but woik veiy well in minoi
flaies and subacute atopic deimatitis.
4. Oial H
1
antihistamines aie useful in ieduc-
ing itching.
FICük£ 2-16 Atopìc dermatìtìs ìo b|ack chì|d: Io||ìcu|ar ||u||||c |o|||cu|+| p+pu|eº ou ||e poº|e||o| |eç.
|o|||cu|+| ec/er+ |º + |e+c||ou p+||e|u ||+| occu|º ro|e corrou|, |u A|||c+u +ud Aº|+u c|||d|eu.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 40
5. Systemic glucocoiticoids should be avoided,
except in iaie instances in adults foi only
shoit couises (iescue tieatment). They aie
widely oveiused. Osteopenia and cataiacts
aie complications. Foi seveie intiactable dis-
ease, piednisone, 60-80 mg daily foi 2 days,
then halving the dose each 2 days foi the
next 6 days. Patients with AD tend to become
dependent on oial glucocoiticoids. Often,
small doses (5-10 mg) make the diffeience
in contiol and can be ieduced giadually to
even 2.5 mg/d, as is often used foi the contiol
of asthma. Intiamusculai glucocoiticoids aie
iisky and should be avoided.
6. UVA-UVB phototheiapy (combination of
UVA plus UVB and incieasing the iadiation
dose each tieatment, with a fiequency of two
to thiee times weekly). Naiiow band UV
(311 nm), PUVA photochemotheiapy also
effective.
7. In seveie cases of adult AD and in noimo-
tensive healthy peisons without ienal dis-
ease cyclospoiine tieatment (staiting dose
5 mg/kg pei day) is indicated when all othei
tieatments fail, but should be monitoied
closely. Tieatment is limited to 3-6 months
because of potential side effects, including
hypeitension and ieduced ienal function.
Blood piessuie should be checked weekly
and chemistiy panels biweekly. Nifedipine
can be used foi modeiate incieases in blood
piessuie.
8. Patients should leain and use stiess manage-
ment techniques.
9. A suggested algoiithm of AD management is
as follows (see Image 2-2):
· Baseline theiapy of diyness with emol-
lients
· Suppiession of mild to modeiate AD by
piolonged topical pimeciolimus oi tacio-
limus and continued emollients
· Supiession of seveie flaies with topical
glucocoiticoids followed by pimecioli-
mus oi taciolimus and emollients
· Oial and topical antibiotics to eliminate
S. aureus
· Hydioxyzine to suppiess piuiitus
Website: |ìì¡.//www.aaJ.org/¡am¡||eìs/et:ema.
|ìm|.
FICük£ 2-1T Chì|dood atopìc dermatìtìs I||º |º + çeue|+||/ed e|up||ou couº|º||uç o| cou||ueu|, |u||+rr+·
|o|, p+pu|eº ||+| +|e e|oº|.e, e\co||+|ed, +ud c|uº|ed.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 41
FICük£ 2-18 Adu|t atopìc dermatìtìs ìo dark skìo Ceue|+||/ed e|up||ou o| |o|||cu|+| p+pu|eº ||+| +|e ro|e
|e+.||, p|çreu|ed ||+u uo|r+| º||u |u + 5!·,e+|·o|d Wor+u o| A|||c+u e\||+c||ou.
IMAC£ 2-2 Ireatmeot a|¿orìthm oI A0.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 42
FAIh0C£N£SIS
A special piedilection of the skin to iespond to
physical tiauma by epideimal hypeiplasia; skin
becomes highly sensitive to touch. The veiy
abnoimal itching hypeiexcitability of licheni-
fied skin aiises in iesponse to minimal exteinal
stimuli that would not elicit an itch iesponse
in noimal skin. Emotional stiess in some cases.
It becomes a habit and may peisist foi months
to yeais, with iesulting maiked lichenification.
A ºpec|+| |oc+||/ed |o|r o| ||c|eu|||c+||ou, occu|·
||uç |u c||curºc|||ed p|+queº.
keºu||º ||or |epe||||.e |u|||uç +ud ºc|+|c||uç.
||c|eu|||c+||ou |º + c|+|+c|e||º||c |e+|u|e o| +|op|c
de|r+||||º, W|e||e| çeue|+||/ed o| |oc+||/ed.
|'C c+u |+º| |o| dec+deº uu|eºº ||e |u|||uç +ud
ºc|+|c||uç +|e º|opped |, ||e+|reu|.
0ccu|º |u |ud|.|du+|º o|de| ||+u 20 ,e+|º, |º ro|e
||equeu| |u Woreu, +ud poºº|||, ro|e ||equeu|
|u Aº|+uº.
IICh£N SIMFI£X Chk0NICüS (|'C) |C|·9 . o93.!

|C|·¹0 . |23
Many patients have AD oi an atopic back-
giound.
Skin symptoms consist of piuiitus, often
in paioxysms. The lichenified skin is like an
eiogenous zone-it becomes a pleasuie (oi-
giastic) to sciatch. Often the aieas on the feet
aie iubbed at night with the heel and the toes.
The iubbing becomes automatic and ieflexive
and an unconscious habit. Most patients with
LSC give a histoiy of itch attacks staiting fiom
minoi stimuli: putting on clothes, iemoving
FICük£ 2-20 Adu|t atopìc dermatìtìs ìo heavì|y
pì¿meoted Asìao skìo I|e|e +|e ru|||p|e p|u||||c
|o|||cu|+| p+pu|eº ||+| +|e + |,p|c+| |e+c||ou p+||e|u |u
A|||c+u +ud Aº|+u º||u.
FICük£ 2-19 Adu|t atopìc dermatìtìs ||c|eu|||·
c+||ou doeº uo| ou|, occu| |u ||e ||ç ||e\u|+| |o|dº |u|
r+, +|ºo +||ec| ||e |+ce |u |||º 5!·,e+|·o|d Wor+u o|
|udoueº|+u e\||+c||ou.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 43
ointments, clothes iubbing the skin; in bed, the
skin becomes waimei and the waimth piecipi-
tates itching.
CIINICAI MANIF£SIAII0N
Skìo Iesìoos A solid plaque of lichenification,
aiising fiom the confluence of small papules;
scaling is minimal except on lowei extiemities
(Fig. 2-21). Lichenified skin is palpably thick-
ened; skin maikings (baiely visible in noimal
skin) aie accentuated and can be seen ieadily.
Excoiiations aie often piesent. Usually dull ied,
latei biown oi black hypeipigmentation, espe-
cially in skin phototypes IV, V, and VI. Round,
oval, lineai (following path of sciatching).
Usually shaiply defined. Isolated single lesion oi
seveial iandomly scatteied plaques. Nuchal aiea
(female) (Fig. 2-21), scalp, ankles, lowei legs,
uppei thighs, exteiioi foieaims, vulva, pubis,
anal aiea, sciotum (See Fig. 35-18), and gioin.
In black skin, lichenification may assume
a special type of pattein-theie is not a solid
plaque, but the lichenification consists instead
of a multitude of small (2- to 3-mm) closely set
papules-i.e., a °folliculai" pattein (as in Fig.
2-16).
0IFF£k£NIIAI 0IACN0SIS
Includes a chionic piuiitic plaque of psoiiasis
vulgaiis, eaily stages of mycosis fungoides, ICD,
ACD, epideimal deimatophytosis.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Hypeiplasia of all compo-
nents of epideimis: hypeikeiatosis, acanthosis,
and elongated and bioad iete iidges. Spongiosis
is infiequent. In the deimis theie is a chionic
inflammatoiy infiltiate.
MANAC£M£NI
Difficult. Repeatedly explain to the patient that
the iubbing and sciatching must be stopped.
It is impoitant to apply occlusive bandages at
night to pievent iubbing. Topical glucocoiti-
coid piepaiations oi tai piepaiations such as
combinations of 5% ciude coal tai in zinc oxide
paste plus class II glucocoiticoids all coveied by
occlusive diessings aie effective foi body aieas
wheie this appioach is feasible (e.g., legs, aims).
Occlusive diessings: topical glucocoiticoids aie
applied to lesion and coveied by an occlusive
(plastic) diessing (like saian wiap). Glucocoi-
ticoids incoipoiated in adhesive plastic tape aie
also veiy effective, left foi 24 houis. Unna Boot:
a gauze ioll diessing impiegnated with zinc ox-
ide paste is wiapped aiound a laige lichenified
aiea such as the calf. The diessing can be left on
foi up to 1 week.
Intialesional tiiamcinolone is often highly
effective in smallei lesions (3 mg/mL; highei
concentiations may cause atiophy). Oial hy-
dioxyzine, 25-50 g at night, may be helpful.
FICük£ 2-21 Iìcheo sìmp|ex chrooìcus Cou·
||ueu|, p+pu|+|, |o|||cu|+| ec/er+, c|e+||uç + p|+que
o| ||c|eu º|rp|e\ c||ou|cuº o| ||e poº|e||o| uec| +ud
occ|p||+| ºc+|p. Coud|||ou |+d |eeu p|eºeu| |o| r+u,
,e+|º +º + |eºu|| o| c||ou|c |u|||uç o| ||e +|e+.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 44
|º o||eu +ººoc|+|ed W||| A| o| occu|º W|||ou| A|.
|| p+||eu|º W||| A| +|e ,ouuçe| +ud |+.e |e+c·
||.||, |o eu.||oureu|+| +||e|çeuº, uou+|op|c ||
p+||eu|º +|e o|de| +ud |+c| |,pe|ºeuº|||.|||eº |o
eu.||oureu|+| +||e|çeuº.
|| º|+||º W||| p|e|c|uç p|u|||uº ||+| |e+dº |o p|c|·
|uç +ud ºc|+|c||uç.
|ore·º|+ped uodu|eº-ºe.e|+| r||||re|e|º |o 2
cr-de.e|op ou º||eº |u W||c| pe|º|º|eu| ||c||uç
+ud ºc|+|c||uç occu| (||ç. 2·22).
|odu|eº +|e o||eu e|oded, e\co||+|ed, +ud ºore·
||reº e.eu u|ce|+|ed +º p+||eu|º d|ç |u|o ||er
W||| ||e|| u+||º.
uºu+||, ru|||p|e ou ||e e\||er|||eº.
|| uºu+||, occu|º |u ,ouuçe| o| r|dd|e·+çe
|er+|eº, W|o o||eu e\||||| º|çuº o| ueu|o||c º||ç·
r+||/+||ou.
|eº|ouº pe|º|º| |o| rou||º +||e| ||e ||+ur+ |+º
|eeu d|ºcou||uued.
I|e+|reu|. |u||+|eº|ou+| |||+rc|uo|oue, occ|uº|.e
d|eºº|uçº W||| ||ç|·po|euc, ç|ucoco|||co|dº. |u
ºe.e|e c+ºeº, ||+||dor|de 50-¹00 rç. w+|c| ou|
|o| cou||+|ud|c+||ouºl |0 ueu|ou||u !00 rç I||
r+, |e |e|p|u|.
FkükIC0 N00üIAkIS (FN) |C|·9 o93.!

|C|·¹0 . |23.¹
FICük£ 2-22 Frurì¿o oodu|arìs \u|||p|e, |||r, e\co||+|ed uodu|eº +||º|uç +| º||eº o| c||ou|c+||, p|c|ed o|
e\co||+|ed º||u. 0||eu occu|||uç |u p+||eu|º W||| +|op, |u| +|ºo W|||ou| ||. I||º |º + !o·,e+|·o|d r+|e W||| n|\ d|ºe+ºe.
ne |+d \k'A ºecoud+|, |u|ec||ou o| p|u||ço uodu|eº. Ce||u||||º o||ç|u+|ed |u p|u||ço uodu|eº |equ|||uç ru|||p|e
|oºp||+||/+||ouº.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 45
IA80kAI0k¥ £XAMINAII0NS
8acterìa| Cu|ture Vesicles of DED aie steiile.
But iule out S. aureus infection.
k0h Freparatìoo Rule out epideimal deima-
tophytosis.
0ermatopatho|o¿y Eczematous inflammation
(spongiosis and intiaepideimal edema) with
intiaepideimal vesicles.
C0ükS£ AN0 Fk0CN0SIS
Recuiient attacks aie the iule. Spontaneous
iemissions in 2-3 weeks. Inteival between at-
tacks is weeks to months. Secondaiy infection
may complicate the couise: pustules, ciusts,
cellulitis, lymphangitis, and painful lymphade-
nopathy. Disabling because of seveie, fiequently
iecuiiing outbieaks.
MANAC£M£NI
Wet 0ressìo¿ Foi vesiculai stage: Buiow wet
|,º||d|o||c ec/er+ |º + ºpec|+| .eº|cu|+| |,pe o|
|+ud +ud |oo| de|r+||||º.
Au +cu|e, c||ou|c, o| |ecu||eu| de|r+|oº|º o| ||e
||uçe|º, p+|rº, +ud ºo|eº.
'uddeu ouºe| o| r+u, deep·ºe+|ed p|u||||c, c|e+|
'|+p|oc+·|||e¨ .eº|c|eº (||ç. 2·2!).
|+|çe |u||+e +ud |+c|e||+| |u|ec||ou c+u occu|.
|+|e|, ºc+||uç ||ººu|eº +ud ||c|eu|||c+||ou.
',¤¤¤,¤º |orp|o|,\, .eº|cu|+| p+|r+| ec/er+.
|C|·9 . 105.3¹

|C|·¹0 . |!0.¹
0¥ShI0k0IIC £CI£MAI0üS 0£kMAIIIIS (0£0)
diessings. Laige bullae diained with a punctuie
but not unioofed.
Fìssures Topical application of flexible col-
lodion.
C|ucocortìcoìds
TvpIcu| High-potency glucocoiticoids with
plastic occlusive diessings foi 1 to maximum
of 2 weeks.
1ntru|esIvnu| 1n¡ectIvn Tiiamcinolone, 3 mg/
mL. Veiy effective foi small aieas of involve-
ment.
SystemIc In seveie cases, a shoit, tapeied
couise of piednisone can be given: 70 mg/d,
tapeiing by 10 oi 5 mg/d ovei 7 oi 14 days.
Systemìc Aotìbìotìc Foi suspected (localized
pain) oi documented secondaiily infected le-
sions (usually S. aureus, less commonly gioup
A stieptococcus).
FüvA (See pa¿e 68) Oial oi topical as °soaks."
Successful in many patients if given ovei pio-
longed peiiods of time and woith tiying, espe-
cially in seveie cases.
FICük£ 2-23 0yshìdrotìc ectematous dermatìtìs Cou||ueu| |+p|oc+·|||e .eº|c|eº +ud c|uº|ed (e\co||+|ed)
e|oº|ouº ou ||e do|ºur o| ||uçe|º +ud ||uçe| We|º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 46
£FI0£MI0I0C¥
Two peaks in incidence: young adulthood and
old age. Fall and wintei.
FAIh0C£N£SIS
Unknown. Unielated to atopic diathesis; IgE
levels noimal. Incidence peaks in wintei, when
xeiosis is maximal. S. aureus often piesent but
pathogenic significance not pioven.
CIINICAI MANIF£SIAII0N
Skìo Symptoms Piuiitus, often intense.
Skìo Iesìoos Closely giouped, small vesicles
and papules that coalesce into plaques (Fig.
2-24A), often moie than 4 to 5 cm in diametei,
with an eiythematous base with distinct boi-
deis. Plaques may become exudative and ciust
(Fig. 2-24B). Excoiiations secondaiy to sciatch-
ing. Diy scaly plaques that may be lichenified.
Round oi toín-s|a¡eJ (Fig. 2-24A), hence the
adjective nummu|ar (Latin: nummu|arís, °like
a coin"). Maigins often moie pionounced than
centei.
DIstrIhutIvn Regional clusteis of lesions (e.g.,
on legs oi tiunk) oi geneialized, scatteied.
Lowei legs (oldei men), tiunk, hands and fin-
geis (youngei females).
0IFF£k£NIIAI 0IACN0SIS
Sca|ìo¿ F|aques Epideimal deimatophytosis,
ICD oi ACD, psoiiasis, eaily stages of mycosis
fungoides, impetigo, familial pemphigus.
|urru|+| ec/er+ |º + c||ou|c, p|u||||c, |u·
||+rr+|o|, de|r+||||º occu|||uç |u ||e |o|r
o| co|u·º|+ped p|+queº corpoºed o| ç|ouped
ºr+|| p+pu|eº +ud .eº|c|eº ou +u e|,||er+|ouº
|+ºe.
|| |º eºpec|+||, corrou ou ||e e\||er|||eº du||uç
W|u|e| rou||º, o||eu ºeeu |u +|op|c |ud|.|du+|º,
',¤¤¤,¤. ||ºco|d ec/er+, r|c|o||+| ec/er+.
|C|·9 . o92.9

|C|·¹0 . |!0.9
NüMMüIAk £CI£MA (N£)
IA80kAI0k¥ £XAMINAII0NS
8acterìa| Cu|ture Rule out S. aureus infection.
0ermatopatho|o¿y Subacute inflammation
with acanthosis and spongiosis.
C0ükS£ AN0 Fk0CN0SIS
Chionic. Lesions last fiom weeks to months.
Often difficult to contiol even with potent topi-
cal glucocoiticoid piepaiations.
MANAC£M£NI
Skìo hydratìoo °Moistuiize" involved skin af-
tei bath oi showei with hydiated petiolatum oi
othei moistuiizing cieam.
C|ucocortìcoìds TvpIcu| PrepurutIvns
Classes I and II applied twice daily until
lesions have iesolved. Steioid impiegnated
tape. Inìra|esíona| tiiamcinolone, 3 mg/mL.
Crude Coa| Iar 2-5% ciude coal tai ointment
daily. May be combined with glucocoiticoid
piepaiation. Tai baths aie useful in patients
with iefiactoiy lesions.
Systemìc Iherapy Systemic antibiotics if S.
aureus is piesent.
FüvA or üv8 311-om Iherapy Veiy effective.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 4T
FICük£ 2-24 Nummu|ar ectema  ||u||||c, |ouud, uurru|+| (co|u·º|+ped) p|+queº W||| e|,||er+, ºc+|eº,
+ud c|uº|º ou ||e |o|e+|r.  A c|oºe·up o| + |eº|ou |u +uo||e| p+||eu| |e.e+|º ||+| |||º |u||+rr+|o|, p|+que couº|º|º
o| cou||ueu| p+pu|o.eº|cu|+| |eº|ouº ||+| oo/e + ºe|ouº ||u|d +ud |e+d |o c|uº||uç +ud +|e uºu+||, ,e||oW.


FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 48
Au o||eu uu|ecoçu|/ed çeue|+||/ed p|u||||c de|·
r+||||º d||ec||, |e|+|ed |o + p||r+|, de|r+||||º
e|ºeW|e|e.
|o| e\+rp|e, + p+||eu| W||| .euouº º|+º|º de|r+·
||||º ou ||e |oWe| |eçº r+, de.e|op p|u||||c, º,r·
re|||c, ºc+||e|ed, e|,||er+|ouº, r+cu|op+pu|+|,
o| p+pu|o.eº|cu|+| |eº|ouº ou ||e ||uu|, |o|e+|rº,
|||ç|º, o| |eçº.
I|eºe pe|º|º| +ud ºp|e+d uu||| ||e |+º|c uude||,·
|uç p||r+|, de|r+||||º |º cou||o||ed.
'|r||+||,, +u|oºeuº|||/+||ou r+, occu| +º +u '|d¨
|e+c||ou |u |u||+rr+|o|, ||ue+ ped|º +ud r+u|·
|eº|º +º + d,º||d|oº||o|r, .eº|cu|+| e|up||ou ou
||e |ee| +ud |+udº (||ç. 2·25) +ud p+pu|o.eº|cu·
|+| ec/er+|o|d |eº|ouº ou ||e ||uu|.
I|e p|euoreuou |eºu||º ||or ||e |e|e+ºe o|
c,|o||ueº |u ||e p||r+|, de|r+||||º, +º + |eºu||
o| ºeuº|||/+||ou. I|eºe c,|o||ueº c||cu|+|e |u ||e
||ood +ud |e|ç||eu ||e ºeuº|||.||, o| ||e d|º|+u|
º||u +|e+º.
I|e d|+çuoº|º o| +u|oºeuº|||/+||ou de|r+||||º |º o|·
|eu ¤¤º| |¤:, |.e., ||e d|º|+u| e|up||ou d|º+ppe+|º
W|eu ||e p||r+|, de|r+||||º |º cou||o||ed.
0|+| ç|ucoco|||co|dº |+º|eu ||e d|º+ppe+|+uce o|
||e |eº|ouº.
AüI0S£NSIIIIAII0N 0£kMAIIIIS |C|·9 . o92.9

|C|·¹0 . |!0.9
A .e|, corrou c||ou|c de|r+|oº|º c|+|+c|e||/ed
|, |edueºº +ud ºc+||uç +ud occu|||uç |u |eç|ouº
W|e|e ||e ºe|+ceouº ç|+udº +|e roº| +c||.e, ºuc|
+º ||e |+ce +ud ºc+|p, ||e p|eº|e|u+| +|e+, +ud |u
||e |od, |o|dº. \||d ºc+|p '| c+uºeº ||+||uç, |.e.,
d+ud|u||.
Ceue|+||/ed '|, |+||u|e |o ||||.e, +ud d|+|||e+ |u
+u |u|+u| º|ou|d |||uç |o r|ud |e|ue| d|ºe+ºe W|||
+ .+||e|, o| |rruuode||c|euc, d|ºo|de|º.
',¤¤¤,¤º. 'C|+d|e c+p¨ (|u|+u|º), p||,||+º|º º|cc+
(d+ud|u||).
S£80kkh£IC 0£kMAIIIIS (S0) |C|·9 . o09.¹

|C|·¹0 . |2¹.9
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset Infancy (within the fiist months),
pubeity, most between 20 and 50 yeais oi
oldei.
Sex Moie common in males.
Iocìdeoce 2 to 5% of the population.
Fredìsposìo¿ aod £xacerbatìo¿ Factors In
immunocompetent patients theie is often a
heieditaiy diathesis, the so-called seboiiheic
state, with maiked seboiihea and maiginal
blephaiitis. May be associated with psoiiasis
as a piepsoiiasis state in which the patient
latei develops psoiiasis; in some patients a mix
of lesions (supeificial scales on the scalp and
eyebiows and polycyclic scaling patches on the
tiunk) suggests the use of the teim se|orr|íasís .
Theie is ieputedly an incieased incidence in
Paikinson disease and facial paialysis. Also,
some neuioleptic diugs aie possibly a factoi,
but the disease is so common that this has not
been pioved. Emotional stiess is a putative fac-
toi in flaies. HIV-infected individuals have an
incieased incidence, and seveie intiactable SD
should be a clue to the existence of HIV disease
(see also Section 31).
FAIh0C£N£SIS
Ma|asse:ía [ur[ur is said to play a iole in the patho-
genesis, and the iesponse to topical ketoconazole
and selenium sulfide is some indication that this
yeast may be pathogenic; also the fiequency of
SD in immunosuppiessed patients (HIV/AIDS,
caidiac tiansplants). SD-like lesions aie seen in
nutiitional deficiencies such as zinc deficiency
(as a iesult of IV alimentation) and expeiimental
niacin deficiency and in Paikinson disease (in-
cluding diug-induced). SD develops in expeii-
mental pyiidoxine deficiency in humans.
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos Giadual onset.
Seasooa| varìatìoos Some patients aie woise
in wintei in a diy, indooi enviionment. Sun-
light exposuie causes SD to flaie in a few
S£CII0N 2 EC/E\A/|Ek\AI|I|' 49
patients and piomotes impiovement of the
condition in otheis.
Skìo Symptoms Piuiitus is vaiiable, often in-
cieased by peispiiation.
Skìo Iesìoos Oiange-ied oi giay-white skin,
often with °gieasy" oi white diy scaling macules,
papules of vaiying size (5-20 mm), oi patches,
iathei shaiply maiginated (Fig. 2-26). Sticky
ciusts and fissuies aie common in the folds
behind the exteinal eai. On the scalp theie is
mostly maiked scaling (°dandiuff "), diffuse
involvement of scalp. Scatteied, disciete on the
face and tiunk. Nummulai, polycyclic, and even
annulai on the tiunk .
0ìstrìbutìoo aod Major Iypes oI Iesìoos (8ased
oo Ioca|ìtatìoo aod A¿e) HuIry Areus v]
Heud Scalp, eyebiows, eyelashes (blephaiitis),
beaid (folliculai oiifices); ciadle cap: eiythema
and yellow-oiange scales and ciusts on the scalp
in infants .
Fuce The flush (°butteifly") aieas, on foiehead
(°coiona seboiihoica"), nasolabial folds, eye-
biows, glabella (Fig. 2-26). The eiythema of SD
is often oveilooked and thought to be the flush-
ing of iosacea. SD does not iespond to tieat-
ment of iosacea. Eais: ietioauiiculai, meatus.
Trun| Simulating lesions of pityiiasis io-
sea oi pityiiasis veisicoloi; yellowish-biown
patches ovei the steinum common.
Bvdy Fv|ds Axillae, gioins, anogenital aiea,
submammaiy aieas, umbilicus, and diapei aiea
in infants (Fig. 2-27)-piesents as a dif-
fuse, exudative, shaiply maiginated, biightly
eiythematous eiuption; eiosions and fissuies
common.
GenItu|Iu Often with yellow ciusts and pso-
iiasifoim lesions.
0IACN0SIS[0IFF£k£NIIAI 0IACN0SIS
Usually made on clinical ciiteiia.
ked Sca|y F|aques Cvmmvn Mild psoiiasis
vulgaiis (sometimes may be indistinguishable),
impetigo (iule out by smeais foi bacteiia), dei-
matophytosis (tinea capitis, tinea facialis, tinea
coipoiis), pityiiasis veisicoloi, inteitiiginous
candidiasis (KOH: iule out deimatophytes and
yeasts), subacute lupus eiythematosus (iule
out by biopsy), °seboiiheic" papules in sec-
ondaiy syphilis (daikfield: iule out Tre¡onema
¡a||íJum ).
Rure Langeihans cell histiocytosis (occuis in
infants, often associated with puipuia), acio-
deimatitis enteiopathica, zinc deficiency, pem-
phigus foliaceus, glucagonoma syndiome.
IA80kAI0k¥ SIü0I£S
0ermatopatho|o¿y Focal paiakeiatosis,
with few neutiophils, modeiate acanthosis,
spongiosis (inteicellulai edema), nonspecific
FICük£ 2-25 Autoseosìtìtatìoo
dermatìtìs ("ìd" reactìoo): derma-
tophytìd \eº|c|eº +ud |u||+e ou ||e
||uçe| +ud ||e |+|e|+| |oo| o| + 2¹·,e+|·
o|d |er+|e. Bu||ouº (|u||+rr+|o|,) ||ue+
ped|º W+º p|eºeu| +ud W+º +ººoc|+|ed
W||| de|r+|op|,||d |e+c||ou. ||edu|ºoue
W+º ç|.eu |o| 2 Wee|º, p|u|||uº +ud
.eº|cu|+||ou |eºo|.ed.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 50
inflammation of the deimis. A chaiacteiistic fea-
tuie is neutiophils at the tips of the dilated fol-
liculai openings, which appeai as ciusts/scales.
C0ükS£ AN0 Fk0CN0SIS
SD is veiy common, affecting the majoiity of
individuals at some time duiing life. The condi-
tion impioves in the summei and flaies in the
fall. Recuiiences and iemissions, especially on
the scalp, may be associated with alopecia in
seveie cases. Infantile and adolescent SD disap-
peais with age. Seboiiheic eiythiodeima may
occui. Se|orr|eít eryì|roJerma wíì| Jíarr|ea
anJ [aí|ure ìo ì|rí·e (Leíner Jísease) ín ín[anìs
ís assotíaìeJ wíì| a ·aríeìy o[ ímmunoJe[ítíenty
JísorJers ínt|uJíng Je[etìí·e yeasì o¡soní:aìíon,
CJ Je[ítíenty, se·ere tom|íneJ ímmunoJe[í-
tíenty, |y¡ogammag|o|u|ínemía, anJ |y¡erím-
munog|o|u|ínemía.
MANAC£M£NI
Requiies initial theiapy followed by chionic
maintenance theiapy. Topical glucocoiticoid
piepaiations aie effective but can cause atiophy
and eiythema and telangiectasia, especially on
the face, oi initiation/exaceibation of peiioial
deimatitis oi iosacea. UV iadiation is benefi-
cial foi many individuals. Topical calcineuiin
inhibitois aie highly effective.
Ioìtìa| Iopìca| Iherapy
Sca|p Adu|ts Effective ovei-the-countei
(OTC) s|am¡oos containing selenium sulfide,
zinc pyiithione, aie helpful. By piesciiption
(U.S.), 2% ketoconazole shampoo, used in-
itially to tieat and subsequently to contiol
the symptoms; lathei can be used on face and
chest duiing showei. Tai shampoos (OTC) aie
equally effective in many patients. Low-potency
g|utotorìítoíJ solution, lotion, oi gels following
FICük£ 2-26 Seborrheìc derma-
tìtìs oI Iace: adu|t-type E|,||er+
+ud ,e||oW·o|+uçe ºc+||uç +uuu|+| o|
||e |o|e|e+d, c|ee|º, u+ºo|+||+| |o|dº,
+ud c||u. 'c+|p +ud |e||o+u||cu|+| +|e+º
We|e +|ºo |u.o|.ed.
S£CII0N 2 EC/E\A/|Ek\AI|I|' 51
a medicated shampoo (ketoconazole oi tai) foi
moie seveie cases. Pimeciolimus, 1% cieam, is
veiy beneficial.
1n]unts Foi ciadle cap, iemoval of ciusts
with waim olive oil compiesses, followed by
baby shampoo, 2% ketoconazole shampoo, and
application of 1-2.5% hydiocoitisone cieam,
2% ketoconazole cieam, 1% pimeciolimus
cieam.
Face aod Iruok Keìotona:o|e s|am¡oo , 2%.
Glucocoiticoid cieam and lotions: initially 1 oi
2.5% hydiocoitisone cieam, 2% ketoconazole
cieam, 1% pimeciolimus cieam, 0.03 oi 0.1%
taciolimus ointment.
More ¡oìenì g|utotorìítoíJ |oìíons (e.g., clo-
betasol piopionate) may be used foi íníìía|
contiol and aie used along with the medicated
shampoos.
£ye|ìds Gentle iemoval of the ciusts in the
moining with a cotton ball dipped in di-
luted baby shampoo. Apply 10% sodium sul-
facetamide in a suspension containing 0.2%
piednisolone and 0.12% phenylephiine (use
cautiously because it contains glucocoiticoids).
Sodium sulfacetamide ointment alone is also
effective, as is 2% ketoconazole cieam, 1%
pimeciolimus cieam, oi 0.03% taciolimus oint-
ment.
Iotertrì¿ìoous Areas Keìotona:o|e, 2% ; if un-
contiolled with these tieatments, Castellani
paint foi deimatitis of the body folds is of-
ten veiy effective, but staining is a pioblem.
Pimeciolimus cieam, 1%; taciolimus ointment,
0.03 oi 0.1%.
Systemìc Iherapy
In seveie cases, 13- tís ietinoic acid oially, 1 mg/
kg, is highly effective. Contiaception should be
used in females of child-beaiing age. In mildei
cases, itiaconazole 100 twice daily foi 2 weeks is
also effective.
Maìoteoaoce Iherapy
Ketoconazole 2% shampoo; tai shampoos may
be equally effective; ketoconazole cieam. If
these do not woik, then the old °standaid," 3%
sulfui piecipitate and 2% salicylic acid in an
oil-in-watei base is effective; this must be piop-
eily compounded. Also, 1-2.5% hydiocoitisone
cieam daily will woik, but patients should be
monitoied foi signs of atiophy. 1% pimecioli-
mus cieam and 0.03% taciolimus ointment aie
safe and effective.
FICük£ 2-2T Seborrheìc dermatìtìs: ìoIaotì|e-type E|,||er+ +ud o|+uçe ºc+|eº +ud c|uº||uç |u ||e
d|+pe| |eç|ou o| +u |u|+u|. I||º |º d||||cu|| |o d|º||uçu|º| |u ||e d|+pe| |eç|ou ||or pºo||+º|º +ud Cc¤!·!c |+º |o |e
|u|ed ou| |, K0n.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 52
A corrou p|u||||c de|r+||||º ||+| occu|º eºpe·
c|+||, |u o|de| pe|ºouº, |u ||e W|u|e| |u |erpe|+|e
c||r+|eº-|e|+|ed |o ||e |oW |ur|d||, o| |e+|ed
|ouºeº.
I|e º||eº o| p|ed||ec||ou +|e ||e |eçº (||ç. 2·23),
+|rº, +ud |+udº |u| +|ºo ||e ||uu|.
||,, 'c|+c|ed,¨ ºupe|||c|+||, ||ººu|ed º||u W|||
º||ç|| ºc+||uç.
I|e |uceºº+u| p|u|||uº c+u |e+d |o ||c|eu|||c+||ou,
W||c| c+u e.eu pe|º|º| W|eu ||e eu.||oureu|+|
coud|||ouº |+.e |eeu co||ec|ed.
I|e d|ºo|de| |eºu||º ||or |oo ||equeu| |+|||uç
|u |o| ºo+p, |+||º o| º|oWe|º +ud/o| |u o|de|
pe|ºouº ||.|uç |u |oorº W||| + ||ç| eu.||oureu|+|
|erpe|+|u|e +ud |oW |e|+||.e |ur|d||,.
\+u+çereu|. +.o|d|uç o.e||+|||uç W||| ºo+p,
eºpec|+||, |u| |+||º, +ud |uc|e+º|uç ||e +r||eu|
|ur|d||, |o >50°, |, uº|uç |oor |ur|d|||e|º,
+|ºo uº|uç |ep|d W+|e| |+||º cou|+|u|uç |+|| o||º
|o| |,d|+||ou, |o||oWed |, |rred|+|e |||e|+| +p·
p||c+||ou o| ero|||eu| o|u|reu|º, ºuc| +º |,d|+|ed
pe||o|+|ur. || º||u |º |u||+red, uºe red|ur·po·
|euc, ç|ucoco|||co|d o|u|reu|º, +pp||ed |W|ce d+||,
uu||| ||e ec/er+|ouº corpoueu| |+º |eºo|.ed.
',¤¤¤,¤. Ec/er+ :·c¡±-|- (||euc| :·c¡±-|-
'r+||ed W||| c|+c|º,¨ ºuc| +º |u o|d c||u+ +ud
ce|+r|c |||e).
ASI£AI0IIC 0£kMAIIIIS |C|·9 . o92.39

|C|·¹0 . |!0.9
FICük£ 2-28 Asteatotìc dermatìtìs |u |||º o5·,e+|·o|d r+u |eº|ouº |+.e co+|eºced |o |u.o|.e ||e eu|||e º||u
o| ||e |oWe| |eç. I|e e|,||er+|ouº, ºc+||uç |eº|ouº +|e e\||ere|, p|u||||c.
53
CIASSIFICAII0N
Psoriasis vulgaris
Acute guttate
Chionic stable plaque
Palmoplantai
Inveise
S E C I | 0 N 5
FS0kIASIS
|ºo||+º|º +||ec|º ¹.5-2° o| ||e popu|+||ou |u
Weº|e|u couu|||eº. wo||d W|de occu||euce.
A c||ou|c d|ºo|de| W||| po|,çeu|c p|ed|ºpoº|||ou
+ud |||ççe||uç eu.||oureu|+| |+c|o|º ºuc| +º
|+c|e||+| |u|ec||ou, ||+ur+, o| d|uçº.
'e.e|+| c||u|c+| e\p|eºº|ouº. I,p|c+| |eº|ouº +|e
c||ou|c, |ecu|||uç, ºc+|, p+pu|eº +ud p|+queº.
|uº|u|+| e|up||ouº +ud e|,|||ode|r+ occu|.
C||u|c+| p|eºeu|+||ou .+||eº +rouç |ud|.|du+|º,
||or ||oºe W||| ou|, + |eW |oc+||/ed p|+queº |o
||oºe W||| çeue|+||/ed º||u |u.o|.ereu|.
|ºo||+||c +|||||||º occu|º |u ¹0-25° o| ||e
p+||eu|º.
I|e p+||oçeueº|º |º de|e|r|ued |, + po|,çeu|c
||+|| W||| +u ouço|uç I ce||-d||.eu +u|o|e+c||.e
|rruue |eºpouºe.
Psoriatic erythroderma
Pustular psoriasis
Pustulai psoiiasis of von Zumbusch
Palmoplantai pustulosis
Aciodeimatitis continua
FS0kIASIS vüICAkIS |C|·9 . o9o.¹

|C|·¹0 . | +0.0 
£FI0£MI0I0C¥
A¿e oI 0oset Ear|y : Peak incidence occuis at
22.5 yeais of age (in childien, the mean age of
onset is 8 yeais). Laìe : Piesents about age 55.
Ear|y onseì piedicts a moie seveie and long-last-
ing disease, and theie is usually a positive family
histoiy of psoiiasis.
Iocìdeoce Psoiiasis affects 1.5-2% of the pop-
ulation in westein countiies. In the United
States, theie aie 3 to 5 million peisons with
psoiiasis. Most have localized psoiiasis, but ap-
pioximately 300,000 peisons have geneialized
psoiiasis.
Sex Equal incidence in males and females.
kace Low incidence in West Afiicans, Japa-
nese, and Inuits; veiy low incidence oi absence
in Noith and South Ameiican Indians.
heredìty Polygenic tiait. When one pai-
ent has psoiiasis, 8% of offspiing develop
psoiiasis; when both paients have psoiiasis,
41% of childien develop psoiiasis. HLA types
most fiequently associated with psoiiasis aie
HLA- B13, -B17,-Bw57, and, most impoitantly,
HLA-Cw6, which piesents antigens to CD8-
T cells and is thus a candidate foi functional
involvement.
Irì¿¿er Factors P|ysíta| ìrauma (Koebnei
phenomenon) is a majoi factoi in eliciting
lesions; iubbing and sciatching stimulate the
psoiiatic piolifeiative piocess. In[etìíons : acute
stieptococcal infection piecipitates guttate pso-
iiasis. Sìress : a factoi in flaies of psoiiasis is
said to be as high as 40% in adults and highei
in childien. Drugs : systemic glucocoiticoids,
oial lithium, antimalaiial diugs, inteifeion,
and -adieneigic blockeis can cause flaies in
existing psoiiasis and cause a psoiiasifoim
diug eiuption. À|to|o| íngesìíon is a putative
tiiggei factoi.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 54
FAIh0C£N£SIS
The most obvious abnoimalities in psoiiasis
aie (1) an alteiation of the cell kinetics of
keiatinocytes with a shoitening of the cell
cycle fiom 311 to 36 h, iesulting in 28 times
the noimal pioduction of epideimal cells, and
(2) CD8- T cells, which aie the oveiwhelming
T cell population in lesions. The epideimis
and deimis ieact as an integiated system: the
desciibed changes in the geiminative layei of
the epideimis and inflammatoiy changes in the
deimis, which tiiggei the epideimal changes.
Psoiiasis is a T cell-diiven disease. Theie aie
many CD8 - T cells piesent in psoiiatic lesions
suiiounding the uppei deimal blood vessels,
and the cytokine spectium is that of a T
H
1
iesponse. Maintenance of psoiiatic lesions is
consideied an ongoing autoieactive immune
iesponse.
CIINICAI MANIF£SIAII0N
Theie aie two majoi types:
1. Eru¡ìí·e, ín[|ammaìory ìy¡e with multiple
small (guttate oi nummulai) lesions and a
gieatei tendency towaid spontaneous ies-
olution (Figs. 3-1 and 3-2); ielatively iaie
(<2.0% of all psoiiasis); similai to an ex-
anthem: a showei of lesions appeais iathei
iapidly and in young adults, often but not
always following stieptococcal phaiyngitis.
2. C|ronít sìa||e (¡|aque) ¡soríasís (Figs. 3-3;
3-4): Majoiity of patients, with chionic
indolent lesions piesent foi months and
yeais, changing only slowly.
Skìo Symptoms Piuiitus is ieasonably com-
mon, especially in scalp and anogenital pso-
iiasis.
Skìo Iesìoos The classic lesion of psoiiasis
is a shaiply maiginated eiythematous papule
with a silveiy-white scale (Fig. 3-1). Scales aie
lamellai, loose, and easily iemoved by sciatching.
Removal of scale iesults in the appeaiance of
minute blood dioplets ( Àus¡íì: sígn ). Papules
giow to shaiply maiginated plaques with
lamellai scaling (Fig. 3-3) that coalesce to foim
polycyclic oi seipiginous patteins (Fig. 3-4).
May occui anywheie on the body but theie aie
classic piedilection sites (see Image 3-1).
Acute Guttute Type Salmon-pink papules
(guttate: Latin guììa , °diop``), 2.0 mm to 1.0
cm with oi without scales (Figs. 3-1; 3-2) scales
may not be visible but become appaient upon
sciaping. Scatteied disciete lesions, like a iash;
geneially concentiated on the tiunk (Fig. 3-2),
less on the face and scalp, and usually spaiing
palms and soles. Guttate lesions may iesolve
spontaneously within a few weeks but usually
become iecuiient and may evolve into chionic,
stable psoiiasis.
ChrvnIc Stuh|e Type Shaiply maiginated,
dull-ied plaques with loosely adheient, lamel-
lai, silveiy-white scales (Fig. 3-3). Plaques
coalesce to foim polycyclic, geogiaphic lesions
(Fig. 3-4) and may paitially iegiess, iesulting
in annulai, seipiginous, and aicifoim patteins.
Lamellai scaling can easily be iemoved, oi,
when the lesion is extiemely chionic, it ad-
heies tightly to the undeilying inflammatoiy
and infiltiated skin, iesulting in hypeikeia-
tosis that looks like the shell of an oystei
(Fig. 3-5).
0ìstrìbutìoo aod Fredì|ectìoo Sìtes
Acute Guttute Disseminated, geneialized,
mainly tiunk (Fig. 3-2).
ChrvnIc Stuh|e Single lesion oi lesions local-
ized to one oi moie piedilection sites: elbows,
knees, sacialgluteal iegion, scalp, palm/soles
(Image 3-1). Sometimes only iegional involve-
ment (scalp), often geneialized.
Puttern Bilateial, often symmetiic (piedilec-
tion sites); often spaies exposed aieas.
Specìa| Sìtes
Fa|ms aod So|es May be the only aieas in-
volved. Theie is massive silveiy white oi yel-
lowish hypeikeiatosis and scaling, which in
contiast to lesions on the tiunk, is not easily
iemoved (Fig. 3-6). Desquamation of hypei-
keiatosis will, howevei, ieveal an inflammatoiy
plaque at the base that is always shaiply demai-
cated (Fig. 3-7). Theie may be ciacking and
painful fissuies and bleeding.
Sca|p Plaques, shaiply maiginated, with thick
adheient scales (Fig. 3-8). Scatteied disciete
oi diffuse involvement of entiie scalp. Often
veiy piuiitic. Noìe. psoiiasis of the scalp does
not lead to haii loss, even aftei yeais of thick
plaque-type involvement. Scalp psoiiasis may
be pait of geneialized psoiiasis oi coexist with
isolated plaques, oi the scalp may be only site
involved.
Face Uncommonly involved, and when in-
volved, usually associated with a iefiactoiy type
of psoiiasis (Fig. 3-9).
S£CII0N 3 |'0k|A'|' 55
FICük£ 3-1 Fsorìasìs vu|¿arìs |||r+|, |eº|ouº +|e We||·de||ued, |edd|º| o| º+|rou·p|u| p+pu|eº, d|op|||e,
W||| + |ooºe|, +d|e|eu| º||.e|,·W|||e |+re||+| ºc+|e.
FICük£ 3-2 Fsorìasìs vu|¿arìs: buttocks (¿uttate type) ||ºc|e|e, e|,||er+|ouº, ºc+||uç, ºr+|| p+pu|eº
||+| |eud |o co+|eºce, +ppe+||uç +||e| + ç|oup A º||ep|ococc+| p|+|,uç|||º. I|e|e W+º + |+r||, ||º|o|, o| pºo||+º|º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 56
Chrooìc Fsorìasìs oI the Ferìaoa| aod Ceoì-
ta| ke¿ìoos aod oI the 8ody Fo|ds-Ioverse
Fsorìasìs Due to the waim and moist envi-
ionment in these iegions psoiiatic plaques aie
usually not scaly but aie maceiated, often biight
ied and fissuied (Figs. 3-10, 35-7, 35-8). The
shaip demaication peimits distinction fiom
inteitiigo, candidiasis, contact deimatitis, tinea
ciuiis.
Naì|s Fingeinails and toenails fiequently (25%)
involved, especially with concomitant aithiitis
(Fig. 3-11A). Nail changes include pitting, subun-
gual hypeikeiatosis, onycholysis, and yellowish-
biown spots undei the nail plate-the oí| s¡oì
(pathognomonic) (See Figs. 33-8, 33-9).
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y
· Maiked oveiall thickening of the epideimis
(acanthosis) and thinning of epideimis ovei
elongated deimal papillae
· Incieased mitosis of keiatinocytes, fibioblasts,
and endothelial cells
· Paiakeiatotic hypeikeiatosis (nuclei ietained
in the stiatum coineum)
· Inflammatoiy cells in the deimis (lymphocytes
and monocytes) and in the epideimis (lym-
phocytes and polymoiphonucleai cells),
foiming micioabscesses of Munio in the
stiatum coineum.
Sero|o¿y Incieased antistieptolysin titei in
acute guttate psoiiasis with antecedent stiepto-
coccal infection. Sudden onset of psoiiasis may
be associated with HIV infection. Deteimina-
tion of HIV seiostatus is indicated in at-iisk
individuals. Seium uiic acid is incieased in 50%
of patients, usually coiielated with the extent of
the disease; theie is an incieased iisk of gouty
aithiitis. The levels of uiic acid deciease as
theiapy is effective.
Cu|ture Thioat cultuie foi gioup A -hemo-
lytic stieptococcus infection.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Diagnosis is made on clinical giounds.
Acute Cuttate Fsorìasìs Any maculopapulai
diug eiuption, secondaiy syphilis, pityiiasis
iosea.
IMAC£ 3-1 Fredì|ectìoo sìtes oI psorìasìs.
S£CII0N 3 |'0k|A'|' 5T
Sma|| Sca|ìo¿ F|aques Se|orr|eít Jermaìíìís -
may be indistinguishable in sites involved and
moiphology; sometimes teimed se|orr|íasís.
Lít|en sím¡|ex t|ronítus -may complicate pso-
iiasis as a iesult of piuiitus. Psoríasí[orm Jrug
eru¡ìíons -especially beta blockeis, gold, and
methyldopa. Tínea tor¡orís -KOH examina-
tion is mandatoiy, paiticulaily in single lesions.
Mytosís [ungoíJes -scaling plaques can be an
initial stage of mycosis fungoides. Biopsy.
Iar¿e Ceo¿raphìc F|aques Tinea coipoiis, my-
cosis fungoides.
Sca|p Fsorìasìs Seboiiheic deimatitis, tinea
capitis.
Ioverse Fsorìasìs Tinea, candidiasis, inteitiigo,
extiamammaiy Paget disease. C|utagonoma
synJrome -an impoitant diffeiential because
this is a seiious disease; the lesions look like in-
veise psoiiasis. Langeihans cell histiocytosis (see
page 516), Hailey-Hailey disease (see page 105).
Naì|s Onychomycosis. KOH is mandatoiy.
C0ükS£ AN0 Fk0CN0SIS
Acute guttate psoiiasis appeais iapidly, a gen-
eialized °iash.`` Sometimes this type of psoiia-
sis disappeais spontaneously in a few weeks
without any tieatment. Moie often, guttate
psoiiasis evolves into chionic plaque psoiiasis.
This is stable and may undeigo iemission aftei
months oi yeais, iecui, and be a lifelong com-
panion. Chionic geneialized psoiiasis is one
of the °miseiies that beset mankind," causing
shame and embaiiassment and a compiomised
lifestyle. The °heaitbieak of psoiiasis`` is no
joke. As the wiitei John Updike (who himself
has psoiiasis) so poignantly said about being
a peison with psoiiasis, °I am silveiy, scaly.
Puddles of flakes foim wheievei I iest my flesh.
Lusty, though we aie loathsome to love. Keen-
sighted, though we hate to look upon ouiselves.
The name of the disease, spiiitually speaking, is
Humiliation.``
FICük£ 3-3 Fsorìasìs vu|¿arìs: e|bow C||ou|c º|+||e p|+que pºo||+º|º ou ||e e||oW. |u |||º |oc+||ou ºc+|eº
c+u e|||e| +ccuru|+|e |o o,º|e| º|e|||||e |,pe||e|+|oº|º, o| +|e º|ed |u |+|çe º|ee|º |e.e+||uç + |ee|,·|ed |+ºe.
I||º p|+que |+º +||ºeu ||or ||e co+|eºceuce o| ºr+||e|, p+pu|+| |eº|ouº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 58
FICük£ 3-4 Fsorìasìs vu|¿arìs: chrooìc stab|e type \u|||p|e |+|çe ºc+||uç p|+queº ou ||e ||uu|, |u||oc|,
+ud |eçº. |eº|ouº +|e |ouud o| po|,c,c||c +ud cou||ueu| |o|r|uç çeoç|+p||c p+||e|uº. A|||ouç| |||º |º ||e c|+ºº|c+|
r+u||eº|+||ou o| c||ou|c º|+||e p|+que pºo||+º|º, ||e e|up||ou |º º|||| ouço|uç, +º e.|deuced |, ||e ºr+|| çu||+|e
|eº|ouº |u ||e |ur|+| +ud |oWe| |+c| +|e+. I||º p+||eu| W+º c|e+|ed |, +c|||e||u/|u\A cor||u+||ou ||e+|reu|
W||||u + Wee|º.
FICük£ 3-5 Chrooìc p|aque|ìke psorìasìs ìo a 30-year-o|d mao oI Arabìao desceot I|e |,pe|·
|e|+|o||c |o|u, p|+que co.e||uç ||e uude||,|uç |u||+rr+|o|, ||ººue o|ºcu|eº +|| e|,||er+, +ud due |o ||e ||ç|
re|+u|u cou|eu|, ||e ºc+|eº +ppe+| d|||,·ç|+,. I|e |||eçu|+|, º|+|p|, de||ued ||oWu +|e+º +|e poº||u||+rr+|o|,
|,pe|p|çreu|+||ou |u p|e.|ouº pºo||+||c p|+queº.
S£CII0N 3 |'0k|A'|' 59
FICük£ 3-T Fsorìasìs, pa|mar ìovo|vemeot
|ºo||+||c p|+que ou ||e p+|r o| + +5·,e+|·o|d Wor+u,
W||c| ou |||º| º|ç|| ºuççeº|º c||ou|c |||||+||.e de|·
r+||||º. I|e ºp+||uç |u ||e ceu|e| o| ||e p+|r +ud
||e º|+|p de||ue+||ou o| ||e |eº|ou ºuççeº| pºo||+º|º,
W||c| W+º +|ºo p|eºeu| ou o||e| p+||º o| ||e |od,.
FICük£ 3-6 Fsorìasìs vu|¿arìs: so|es E|,||e·
r+|ouº p|+queº W||| |||c|, ,e||oW|º|, |+re||+| ºc+|e
+ud deºqu+r+||ou ou º||eº o| p|eººu|e +||º|uç ou ||e
p|+u|+| |ee|. |o|e º|+|p der+|c+||ou o| ||e |u||+r·
r+|o|, |eº|ou ou ||e +|c| o| ||e |oo|. '|r||+| |eº|ouº
We|e p|eºeu| ou ||e p+|rº.
FICük£ 3-8 Fsorìasìs oI the sca|p I|e|e |º r+ºº|.e corp+c||ou o| |o|u, r+|e||+| ou ||e eu|||e ºc+|p.
|eºqu+r+||ou |+º occu||ed ou ||e |o|e|e+d, W||c| |º +|ºo |u.o|.ed W||| pºo||+º|º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 60
FICük£ 3-9 Fsorìasìs, Iacìa| ìovo|vemeot C|+ºº|c pºo||+||c p|+que ou ||e |o|e|e+d o| + 2¹·,e+|·o|d r+|e
W|o +|ºo |+d r+ºº|.e ºc+|p |u.o|.ereu|.
FICük£ 3-10 Fsorìasìs vu|¿arìs: ìoverse pattero I||º p|c|u|e º|oWº ||e d|||e|euce |e|Weeu p|+que
pºo||+º|º ou uou|||c||ou+| |eç|ouº, ºuc| +º ||e º+c|ur, +ud |u|e||||ç|uouº |eç|ou, ºuc| +º ||e ç|u|e+| |o|d. ne|e ||e
eu.||oureu| |º ro|º| +ud W+|r, W||c| |+c||||+|eº º|edd|uç o| ||e ºc+|, r+|e||+|. |u ||e |u|e|ç|u|e+| |o|d ||e |eº|ou
|º r+ce|+|ed +ud ç|+,|º| ou +u e|,||er+|ouº |+ºe.
S£CII0N 3 |'0k|A'|' 61
FICük£ 3-11 . Fsorìasìs oI the Iìo¿eroaì|s |||º |+.e p|oç|eººed |o e||ou,\|º (|o|eº |u ||e u+|| p|+|eº)
+ud ||e|e |º ||+uº.e|ºe +ud |ouç||ud|u+| ||dç|uç. I||º p+||eu| +|ºo |+º pe||ou,c||+| pºo||+º|º +ud pºo||+||c +|||||||º.
 Fsorìatìc arthrìtìs, |+|e º|+çe |e+d|uç |o +|||||||º ru|||+uº. |eº||uc||ou o| ||e |u|e|p|+|+uçe+| jo|u|º |+º |e+d |o
||e p|euoreuou o| |e|eºcope ||uçe|º +ud |o + ºe.e|e ru|||+||ou o| ||e |+udº W||| couº|de|+||e |uuc||ou+| |rp+||·
reu|. |o| |u|||e| |r+çeº o| u+|| |u.o|.ereu| ºee 'ec||ou !! +ud .


FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 62
C|+|+c|e||/ed |, puº|u|eº, uo| p+pu|eº, +||º|uç ou uo|r+| o| |u||+red, e|,||er+|ouº º||u. IWo |,peº.
FüSIüIAk FS0kIASIS |C|·9 . o9o.¹
A c||ou|c, |e|+pº|uç e|up||ou ||r||ed |o ||e p+|rº
+ud ºo|eº.
|ure|ouº .e|, |,p|c+| º|e|||e, ,e||oW, deep·ºe+|ed
puº|u|eº ||+| e.o|.e |u|o duº|,·|ed c|uº|º.
Couº|de|ed |, ºore +º + |oc+||/ed |o|r o| puº·
|u|+| pºo||+º|º (|+||e|·|,pe) +ud |, o||e|º +º +
ºep+|+|e eu|||,.
FAIM0FIANIAk FüSIüI0SIS |C|·9 . o9o.¹

|C|·¹0 . | +0.! 
£FI0£MI0I0C¥
Iocìdeoce Low as compaied to psoiiasis vul-
gaiis.
A¿e oI 0oset 50 to 60 yeais. Moie common in
females (4:1).
CIINICAI MANIF£SIAII0N
Symptoms Stinging, buining itching. Eiup-
tions come and go, in waves.
Skìo Iesìoos Pustules in stages of evolution,
2-5 mm, deep-seated, yellow, develop into
dusky-ied macules and ciusts; piesent in aieas
of eiythema and scaling oi noimal skin (Fig.
3-12). Limited to palms and soles, may be only
a localized patch on the sole oi hand, oi involve
both hands and feet with a piedilection of
thenai and hypothenai, flexoi aspects of fingeis,
heels, and insteps; acial poitions of the fingeis
and toes usually spaied.
0IFF£k£NIIAI 0IACN0SIS
Conditions confined to palms and soles. Epi-
deimal deimatophytosis (tinea manus, tinea
pedis), dyshidiotic eczematous deimatitis, iiii-
tant oi alleigic contact deimatitis, heipes simplex
viius (HSV) infection (if localized to one site).
IA80kAI0k¥ £XAMINAII0NS
k0h Freparatìoos To exclude deimatophytosis.
8acterìa| or vìra| Cu|ture To exclude Sìa¡|y|o-
tottus aureus infection and HSV infection.
0ermatopatho|o¿y Edema and exocytosis of
mononucleai cells that appeai fiist to foim a ves-
icle, and latei myiiads of neutiophils, which foim
a uniloculai spongifoim pustule. Acanthosis.
C0ükS£ AN0 Fk0CN0SIS
Peisistent foi yeais and chaiacteiized by un-
explained iemissions and exaceibations; iaiely
psoiiasis vulgaiis may develop elsewheie.
S£CII0N 3 |'0k|A'|' 63
FICük£ 3-12 Fa|mar pustu|osìs C|e+r,·,e||oW puº|u|eº ||+| +|e p+|||+||, cou||ueu| ou ||e p+|r o| + 23·
,e+|·o|d |er+|e. |uº|u|eº +|e º|e|||e +ud p|u||||c, +ud W|eu ||e, çe| |+|çe|, |ecore p+|u|u|. A| ||e ||re o| |||º
e|up||ou ||e|e W+º uo o||e| e.|deuce o| pºo||+º|º +u,W|e|e e|ºe ou ||e |od,, |u| 2 ,e+|º |+|e| ||e p+||eu| de.e|·
oped c||ou|c º|+||e p|+que pºo||+º|º ou ||e ||uu|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 64
£FI0£MI0I0C¥
Raie, occuis in adults, iaiely in childien.
FAIh0C£N£SIS
Unknown. The fevei and leukocytosis iesult
fiom the ielease of cytokines and chemokines
fiom the skin into the ciiculation. Theie aie
no known piecipitating factois, and the patient
may oi may not have had a stable plaque-type
psoiiasis in the past.
CIINICAI MANIF£SIAII0N
0oset oI Iesìoos The constellation of fieiy-ied
eiythema followed by foimation of pustules oc-
cuis ovei a peiiod of less than 1 day. Waves of
pustules may follow each othei; as one set diies,
anothei appeais.
Skìo Symptoms Maiked buining, tendeiness.
Coostìtutìooa| Symptoms Headache, chills, fe-
veiishness, maiked fatigue, seveie malaise.
Appearaoce oI Fatìeot Fiightened, °toxic.``
vìta| Sì¿os Fast pulse, iapid bieathing, fevei
that may be high.
Skìo Iesìoos Theie is a sequence of buining,
diffuse eiythema followed by the appeaiance
of clusteis of tiny, nonfolliculai, and veiy
supeificial yellowish to whitish pustules that
usually become confluent, foiming ciicinate
lesions and °lakes`` of pus (Figs. 3-13 and
3-14), Nikolsky phenomenon is positive. Re-
moval of the tops of pustules yields supeificial,
oozing eiosions. Ciusting. Once ciusts aie
shed, new ciops of pustules may appeai in the
same site. Pustules aie steiile. The eiuption is
geneialized.
A |||e·|||e+|eu|uç red|c+| p|o||er W||| +u +||up|
ouºe|.
'||u |u.o|.ereu| º|+||º W||| + |u|u|uç ||e|,·|ed
e|,||er+ ||+| ºp|e+dº |u |ou|º W||| p|upo|u|
º|e|||e puº|u|eº +ppe+||uç |u c|uº|e|º.
|e.e|, çeue|+||/ed We+|ueºº, ºe.e|e r+|+|ºe, +ud
|eu|oc,|oº|º +|e |e+|u|eº |u +|roº| e.e|, p+||eu|.
C£N£kAIII£0 ACüI£ FüSIüIAk FS0kIASIS (v0N IüM8üSCh) |C|·9 . o9o.¹

|C|·¹0 . |+0.¹ 
haìr aod Naì|s Nails become thickened, and
theie is onycholysis; subungual °lakes`` of pus
lead to shedding of nails; haii loss of the telogen
defluvium type (see Section 33) may develop in
2 oi 3 months.
Mucous Membraoes Ciicinate desquamation
of the tongue. This is the only foim of psoiiasis
that involves mucous membianes.
0IFF£k£NIIAI 0IACN0SIS
Wìdespread £rythema wìth Fustu|es The
abiupt onset and the typical evolution of eiy-
thema followed by pustulation aie highly chai-
acteiistic. Neveitheless, blood cultuies should
always be obtained because of possible su-
peiinfection and bacteiemia, especially with
S. aureus . Geneialized HSV infection has um-
bilicated pustules, and the Tzanck tests and viial
cultuies establish the diagnosis. Geneialized
pustulai diug eiuptions ¦e.g., aftei fuiosemide,
amoxicillin/clavulanic acid, and othei diugs
(acute geneialized exanthematous pustulosis,
see Section 22)] may be clinically indistinguish-
able, but patients aie less toxic. Psoiiasis cum
pustulatione (see below) has lesions of classic
psoiiasis with pustulation.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Laige spongifoim pustules
iesulting fiom the migiation of neutiophils to
the uppei stiatum malpighi, wheie they aggie-
gate within the inteistices between the degenei-
ated and thinned keiatinocytes.
8acterìa| Cu|ture oI Iìssue Pustules aie steiile.
Rule out S. aureus infection.
hemato|o¿ìc Polymoiphonucleai leukocytosis-
white blood cell count as high as 20,000/µL.
S£CII0N 3 |'0k|A'|' 65
FICük£ 3-13 Ceoera|ìted acute pustu|ar psorìasìs (voo Iumbusch) \u|||p|e puº|u|eº ou ||ç||, e|,||er+·
|ouº º||u. C|oºe·up o| |eº|ouº º|oWº ||+| ||e, +|e .e|, ºupe|||c|+|, c|e+r, W|||e, +ud co+|eºce, |o|r|uç |+|eº o| puº.
FICük£ 3-14 Ceoera|ìted acute pustu|ar psorìasìs (voo Iumbusch) I||º |er+|e p+||eu| W+º |o\|c +ud
|+d |e.e| +ud pe||p|e|+| |eu|oc,|oº|º. I|e eu|||e |od, W+º co.e|ed W||| º|oWe|º o| c|e+r,·W|||e co+|eºc|uç
puº|u|eº ou + ||e|,·|ed |+ºe. '|uce ||eºe puº|u|eº +|e .e|, ºupe|||c|+|, ||e, c+u |e |||e|+||, W|ped o||, W||c| |eºu||º
|u |ed oo/|uç e|oº|ouº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 66
C0ükS£ AN0 Fk0CN0SIS
These patients aie often biought to the emeig-
ency iooms of hospitals, and theie is the question
of oveiwhelming bacteiemia until a deima-
tologist is consulted and the blood cultuies aie
shown to be negative. Relapses and iemissions
may occui ovei a peiiod of yeais. In the eldeily
piognosis is guaided if not tieated. May follow,
evolve into, oi be followed by psoiiasis vulgaiis.
SF£CIAI I¥F£S
Impetì¿o herpetìIormìs This is von Zumbusch
pustulai psoiiasis in a piegnant woman with
hypocalcemia, leading to tetanic seizuies.
Aoou|ar Iype This type of pustulai psoiia-
sis occuis in childien, with less consitutional
symptoms.
Fsorìasìs cum Fustu|atìooe (psoiiasis with
postulation) This is maltieated stable plaque
psoiiasis wheie pustulation of psoiiatic lesions
and the suiiounding noimal skin occuis ,
usually aftei iiiitating topical tieatment (e.g.,
anthialin) oi systemic glucocoiticoids; may
become geneialized but patients aie usually
nontoxic.
Acrodermatìtìs Cootìoua oI ha||opeau This is a
chionic iecuiient pustulation of nail folds, nail
bed, and distal fingeis leading to loss of nails. It
can occui alone oi in association with pustulai
psoiiasis of von Zumbusch.
FICük£ 3-15 Acrodermatìtìs cootìoua o| n+||ope+u W||| +c|+| puº|u|e |o|r+||ou, ºu|uuçu+| |+|eº o| puº,
+ud deº||uc||ou o| u+|| p|+|eº. I||º r+, |e+d |o pe|r+ueu| |oºº o| u+||º +ud ºc+|||uç.
S£CII0N 3 |'0k|A'|' 6T
I¥F£S
1. °Distal"-seionegative, without subcutane-
ous nodules, and involving, asymmetiically, a
few distal inteiphalangeal joints of the hands
and feet: an asymmetiic oligoaithiitis.
2. Enthesitis-inflammation of ligament insei-
tion into bone.
3. Multilating psoiiatic aithiitis with bone eio-
sion and ultimately leading to osteolysis oi
ankylosis.
4. °Axial"-especially involving the sacioil-
iac, hip, and ceivical aieas with ankylosing
spondylitis.
SkIN S¥MFI0MS AN0 SICNS
Swelling, iedness, tendeiness of involved joints
oi site of enthesitis (e.g., inseition of Achil-
les tendon in calcaneus). Dactylitis-sausage
fingeis. May oi may not be associated with pso-
iiasis elsewheie. Often psoiiatic involvement
of fingeitips and peiiungual skin. Massive
nail involvement by psoiiasis is fiequent (Fig.
3-11A).
Aithiitis may lead to aithiitis mutilans: destiuc-
tion of inteiphalangeal joints iesults in telescope
fingeis with mutilation of hand and considei-
able functional impaiiment (Fig. 3-11B).
I||º |º + coud|||ou |u W||c| pºo||+º|º |u.o|.eº
p|+c||c+||, ||e eu|||e º||u +ud |e+dº |o couº|||u·
||ou+| º,rp|orº. || |º + ºe||ouº coud|||ou +ud
|º d|ºcuººed |u 'ec||ou 3.
FS0kIAIIC £k¥Ihk00£kMA |C|·9 . o9o.¹

|C|·¹0 . |+0 
|ºo||+||c +|||||||º |º |uc|uded +rouç ||e ºe·
|oueç+||.e ºpoud,|o+||||op+|||eº, W||c| |uc|ude
+u|,|oº|uç ºpoud,||||º, eu|e|op+|||c +|||||||º, +ud
|e+c||.e +|||||||º.
Aº,rre|||c pe||p|e|+| jo|u| |u.o|.ereu| o| uppe|
e\||er|||eº +ud eºpec|+||, ºr+||e| jo|u|º.
A\|+| |o|r |u.o|.eº .e||e||+| co|uru, º+c|o||||||º.
Aººoc|+|ed W||| \nC c|+ºº | +u||çeuº, W|||e ||eu·
r+|o|d +|||||||º |º +ººoc|+|ed W||| \nC c|+ºº ||
+u||çeuº.
|uc|deuce |º 5-3°. k+|e |e|o|e +çe 20.
!c, |- ¤·-º-¤| (·¤ !9ª ¤| ·¤!·.·!±c|º) +·||¤±|
c¤, .·º·||- ¤º¤··cº·º ·| º¤ º-c·:| |¤· c |c¤·|,
|·º|¤·,
FS0kIAIIC AkIhkIIIS |C|·9 . o9o.0

|C|·¹0 . |+0.5 
MANAC£M£NI 0F FS0kIASIS
Factors IoI|ueocìo¿ Se|ectìoo oI Ireatmeot
1. Age: childhood, adolescence, young adult-
hood, middle age, 60 yeais.
2. Type of psoiiasis: guttate, plaque, palmai
and palmopustulai, geneialized pustulai
psoiiasis, eiythiodeimic psoiiasis.
3. Site and extent of involvement: |ota|í:eJ to
palms and soles, scalp, anogenital aiea, scat-
teied plaques but <5% involvement; genera|-
í:eJ and >30% involvement.
4. Pievious tieatment: ionizing iadiation, sys-
temic glucocoiticoids, photochemotheiapy
(PUVA), cyclospoiine (CS), methotiexate
(MTX).
5. Associated medical disoideis (e.g., HIV
disease).
Ideally all patients with suspected psoiiasis
should be seen at least once by a deimatologist
to establish the diagnosis and select the best
available tieatment iegimen. Localized pso-
iiasis (coveiing <5% of the body suiface) can
be managed by the piimaiy caie physician if a
piopei iegimen is selected. Psoiiasis of all othei
types, especially geneialized psoiiasis, should be
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 68
managed by a deimatologist who has access to
and knowledge of all theiapies, as combinations
and °iotational" theiapy shifting fiom ultiavio-
let to PUVA to MTX oi the °biologicals."
In the following pages management of pso-
iiasis is discussed in the context of types of
psoiiasis, sites, and extent of involvement.
I0CAIII£0 FS0kIASIS
This consists of a limited numbei of chionic
stable psoiiasis plaques (see Fig. 3-3) on the
piedilection sites oi elsewheie. Heie, fiist-line
theiapies aie topical tieatments.
Iruok aod £xtremìtìes
· Topical fluoiinated glucocoiticoids (beta-
methasone valeiate, fluocinolone aceto-
nide, betamethasone piopionate, clobetasol
piopionate) in ointment base applied aftei
the scales aie iemoved by soaking in wa-
tei. Oínìmenì applied to wet skin, coveied
with plastic wiap, left on oveinight. Gluco-
coiticoid-impiegnated tape useful foi small
plaques.
· Hydiocolloid diessing, left on foi 24-48 h, is
effective and pievents sciatching. Duiing the
day, classes I and II fluoiinated glucocoiti-
coid cieams can be used without occlusion.
Patients develop toleiance (tachyphylaxis)
aftei long peiiods. Ca·eaì : Piolonged appli-
cation of the fluoiinated glucocoiticoids leads
to atiophy of the skin, peimanent stiiae,
and unsightly telangiectasia. Clobetasol-17-
piopionate is stiongei and active even with-
out occlusion. To avoid systemic effects of
this class I glucocoiticoid: maximum of 50 g
ointment pei week.
· Foi small plaques ( 4 cm), tiiamcinolone ac-
etonide aqueous suspension 3 mg/mL diluted
with noimal saline is injected into the lesion.
Must be ínìraJerma|. Varníng : Hypopigmen-
tation at the injection site can iesult; this is
moie appaient in biown and black skin but is
ieveisible.
· Topical anthialin piepaiations aie excellent
when used piopeily. Can be veiy iiiitant;
theiefoie follow diiections on the package
inseit with attention to details.
· Vitamin D analogues (calcipotiiene, 0.005%,
ointment and cieam) aie good nonsteioi-
dal antipsoiiatic topical agents and aie not
associated with cutaneous atiophy. Not as po-
tent as class I glucocoiticoids (e.g., clobetasol
piopionate) but can be combined with them.
Calcipotiiene should not be applied to moie
than 40% of the body suiface and not moie
than 100 g pei week to avoid hypeicalcemia.
Topical taciolimus, 0.1%, has efficacy similai
to that of vitamin D analogues.
· Topical pimeciolimus, 1%, is effective in in-
veise psoiiasis and seboiiheic deimatitis-like
psoiiasis of the face and eai canals.
· Tazaiotene (a topical ietinoid, 0.05 and 0.1%
gel) has similai efficacy but can best be com-
bined with class II (medium stiength) topical
glucocoiticoids, as it can cause iiiitation.
· When theie is >10% (palm of the hand · 1%)
involvement with psoiiatic plaques, it is pief-
eiable to combine these topical tieatments
with 311-nm UVB phototheiapy oi PUVA
photochemotheiapy.
Sca|p MI|d Supeificial scaling and lacking
thick plaques: Tai oi ketoconazole shampoos
[o||oweJ |y betamethasone valeiate, 1% lotion;
if iefiactoiy, clobetasol piopionate, 0.05% scalp
application.
Severe Thick, adheient plaques (Fig. 3-8):
Removal of scales fiom plaques befoie active
tieatment by 10% salicylic acid in mineial oil,
coveied with a plastic cap and left on oveinight.
Aftei shedding of scales, fluocinolone cieam oi
lotion with the scalp coveied with plastic oi a
showei cap, left on oveinight oi foi 6 h. When
the thickness of the plaques is ieduced, clobeta-
sol piopionate, 0.05% lotion, oi calcipotiiene
lotion can be used foi maintenance. If unsuc-
cessful oi iapid iecuiience oi if associated with
geneialized psoiiasis, considei systemic tieat-
ment (see below).
Fa|ms aod So|es (Figs. 3-6 and 3-7) Occlusive
diessings with class I topical g|utotorìítoíJs
in petiolatum. If ineffective, PUVÀ ¡|oìot|e-
moì|era¡y, administeied in specially designed
hand-and-foot lighting cabinets that delivei
UVA. PUVÀ °soa|s' : In this tieatment the
hands and feet aie immeised in a solution of
8-methoxypsoialen (10 mg/L of waim watei)
foi 15 min and then exposed to hand and foot
UVA phototheiapy units. Retinoids (acitietin >
isotietinoin) given oially aie effective in iemov-
ing the thick hypeikeiatosis of the palms and
soles; howevei, combination with topical glu-
cocoiticoids oi PUVA (Re-PUVA) is much
moie efficacious. Systemic tieatments should
be consideied.
Fa|mop|aotar Fustu|osìs (Fig. 3-12) The con-
dition is iecalcitiant to tieatment, but peisist-
ence in tieatment can be iewaiding.
PUVA "Svu|s" v] Hunds und Feet (See above)
Ideal foi this condition. Re-PUVA (see below) is
highly efficacious.
S£CII0N 3 |'0k|A'|' 69
TvpIcu| G|ucvcvrtIcvIds, DIthrunv|, und Cvu|
Tur Ineffective. Stiong glucocoiticoids undei
plastic occlusion (e.g., foi the night) may be
effective but do not pievent iecuiiences. MTX
oi CS foi iecalcitiant cases.
Ioverse Fsorìasìs (Fig. 3-10) Initiate theiapy
with ìo¡íta| g|utotorìítoíJs (caution: these aie
atiophy-pione iegions, steioids should be ap-
plied foi only limited peiiods of time); switch
to topical vitamin D deiivatives oi tazaiotene oi
topical taciolimus oi pimeciolimus. Tai baths
oi Castellani paint sometimes useful. If iesistant
oi iecuiient, considei systemic theiapy.
Naì|s (Fig. 3-11; See also Section 33 ) Topi-
cal tieatments of the fingeinails aie unsatisfac-
toiy. Note also that nail psoiiasis may disappeai
spontaneously oi paii passu with successful
tieatment of psoiiasis. Injection of the nail
fold with intiadeimal tiiamcinolone acetonide
(3 mg/mL) effective but painful and impiactical
when all nails aie involved. PUVA photochemo-
theiapy somewhat effective when administeied
in special hand-and-foot lighting units pio-
viding high-intensity UVA. Long-teim systemic
ietinoids (acitietin, 0.5 mg/kg) aie also effec-
tive, as aie systemic MTX and CS theiapy. Since
a diseased nail (plate) cannot be cuied, theiapy
of nails aims at secuiing regrowì| of a noimal
nail plate. It theiefoie depends on the speed of
nail giowth, which is slow and thus iequiies a
long time; this should be taken into account
when consideiing tieatment that may cause
side effects when administeied ovei a piolonged
peiiod of time.
C£N£kAIII£0 FS0kIASIS
Acute, Cuttate Fsorìasìs (Fig. 3-2) Tieat
stieptococcal infection with antibiotics. Topical
tieatment as foi localized psoiiasis. Naiiow-
band UVB iiiadiation most effective. If it fails,
oial PUVA photochemotheiapy (see below).
Ceoera|ìted F|aque-Iype Fsorìasìs (Fig. 3-4)
Peifoimed eithei by office-based deimatologist
oi in a psoiiasis centei wheie all majoi options
aie available: phototheiapy, PUVA, oi systemic
tieatments which aie given as eithei mono- oi
combined oi iotational theiapy. Combination
theiapy denotes the combination of two oi moie
modalities (as in chemotheiapy); iotational
theiapy denotes switching the patient aftei
cleaiing and a subsequent ielapse to anothei
diffeient tieatment. This is done to pievent
cumulative long-teim side effects.
Narrow-8aod üv8 Fhototherapy (311 nm)
Effective only in psoiiasis with veiy thin
plaques; effectiveness is incieased by combi-
nation with topical glucocoiticoids, vitamin D
analogues, tazaiotene, oi topical taciolimus/
pimeciolimus.
0ra| FüvA Fhotochemotherapy Tieatment
consists of oial ingestion of 8-methoxypsoi-
alen (8-MOP) (0.6 mg 8-MOP pei kilogiam
body weight) oi, in some Euiopean countiies,
5-MOP (1.2 mg/kg body weight) and exposuie
to doses of UVA that aie adjusted to the sensi-
tivity of the patient. UVA is given 1 h (8-MOP)
oi 2 h (5-MOP) aftei ingestion of the psoialen,
staiting at a dose of 0.5 to 1 J/cm
2
, adjusted
upwaid foi skin phototype. Alteinatively, pho-
totoxicity testing is done piioi to tieatment,
which peimits a bettei adjustment of the UVA
dose to the individual`s sensitivity to PUVA. The
UVA dose is incieased at successive tieatment
sessions. Tieatments aie peifoimed two oi thiee
times a week oi, with a moie aggiesive piotocol,
foui times a week. Most patients cleai aftei 19 to
25 tieatments, and the amount of UVA needed
ianges fiom 100 to 245 J/cm
2
.
Lvng-term sIde e]]ects : PUVA keiatoses and
squamous cell caicinomas in some patients
who ieceive an excessive numbei of tieatments.
Re-PUVA (see below) ieduces the total numbei
of tieatments.
In patients with iecalcitiant plaque-type pso-
iiasis, acitietin (in males) oi isotietinoin (in
females) may be combined with othei anti-
psoiiatic theiapy, e.g., PUVA, UVB (311 nm),
topical glucocoiticoids, oi anthialin. These
combination modalities ieduce the length of
tieatments as well as the total amount of an-
tipsoiiatic diug necessaiy foi cleaiing. Topi-
cal glucocoiticoids, calcipotiiene ointments,
anthialin, oial MTX, and oial acitietin, com-
bined with eithei PUVA oi 311-nm UVB, aie
all effective in ieducing the dose of one anothei.
0ra| ketìooìds Acitietin, and isotietinoin aie
veiy effective in inducing desquamation but
only modeiately effective in suppiessing pso-
iiatic plaques (an exception is pustulai psoiia-
sis-see below). They aie highly effective when
combined accoiding to established piotocols
with 311-nm UVB oi PUVA (called Re-PUVA).
T|e |aììer ís ín [atì ì|e mosì e[[etìí·e ì|era¡y ìo
Jaìe [or genera|í:eJ ¡|aque ¡soríasís. A com-
bination of PUVA with acitietin (0,75 mg/kg
body weight) is used foi males; foi females,
PUVA is combined with isotietinoin (1 mg/
kg body weight). Contiaception is mandatoiy
duiing tieatment and foi 2 months aftei it is
completed. Combinations of oial ietinoids and
PUVA impiove the efficacy of each and peimit
a ieduction of the dose and duiation of each if
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' T0
iefiactoiy to tieatment. Foi side effects of ietin-
oids, see page 80 and package inseit.
Methotrexate Iherapy Oial MTX is one of the
most effective tieatments and ceitainly the most
convenient tieatment foi geneialized plaque
type psoiiasis. Neveitheless, MTX is a poten-
tially dangeious diug, piincipally because of
livei toxicity that can occui aftei piolonged use.
Also, iesponse is slow and long-teim tieatment
is iequiied. Hepatic toxicity may occui aftei cu-
mulative doses in noimal peisons ( 1.5 g), but
additional iisk factois include a histoiy of oi ac-
tual alcohol intake, abnoimal livei chemistiies,
IV diug use, and obesity. Inasmuch as hepatic
toxicity is ielated to total life dose, this theiapy
should, in geneial, not be given to young pa-
tients who may face many yeais of theiapy.
Schedu|e v] Methvtrerute Therupy wIth the
TrIp|e-Dvse (WeInsteIn) RegImen Piefeiied
by most ovei the single-dose MTX once weekly.
Begin with a test dose of 5 mg (2.5-mg tablet
followed 24 h latei with a second 2.5-mg tablet);
this dose will asceitain whethei theie is a special
sensitivity to MTX. A complete blood count
(CBC), livei function tests, and seium cie-
atinine levels aie obtained befoie stait of tieat-
ment, aftei 1 week, and 2 weeks theieaftei as the
dose of MTX is incieased. One tablet (2.5 mg)
is given eveiy 12 h foi a total of thiee doses,
i.e., 7.5 mg/week (1/1/1 tablet schedule). Some
patients iespond to this dose; if not, the dose is
incieased aftei 2 weeks to 2/2/2, oi 15 mg/week
total dose, to which most patients iespond. This
iegimen achieves an 80% impiovement but
total cleaiing only in some, and highei doses
inciease the iisk of toxicity. Highei doses may
be needed in oveiweight patients. As patients
iespond the dose of MTX can be ieduced by
one oi two tablets peiiodically.
CBC, LIver FunctIvn, und CreutInIne These
have to be monitoied eveiy 3 months. In pa-
tients with noimal livei chemistiies and no
iisk factois piesent, a livei biopsy should be
done aftei a cumulative dose of appioximately
1500 mg MTX; if the post-MTX livei biopsy
is noimal, iepeat livei biopsy should be done
aftei fuithei theiapy with an additional 1000
to 1500 mg MTX. Be awaie of the vaiious diug
inteiactions with MTX.
Cyc|osporìoe
1
CS tieatment is highly effective
at a dose of 3-5 mg/kg pei day. As the patient
iesponds, the dose is tapeied to the lowest
effective maintenance dose. Monitoiing blood
piessuie and seium cieatinine is mandatoiy be-
cause of the known nephiotoxicity of the diug.
CS s|ou|J |e em¡|oyeJ on|y ín ¡aìíenìs wíì|ouì
rís| [atìors .
Moooc|ooa| Aotìbodìes aod Fusìoo Froteìos
2
(so-called biologicals) Some of these pioteins,
specifically taigeted to pathogenically ielevant
ieceptois on T cells oi to cytokines, have been
appioved and moie aie being developed. They
should be employed only by specifically tiained
deimatologists who aie familiai with the dos-
age schedules, diug inteiactions, and shoit- oi
long-teim side effects.
A|e]ucept is a human lymphocyte function-
associated antigen (LFA)-3-IgG1 fusion piotein
that pievents inteiaction of LFA-3 and CD2.
CD2 is upiegulated in memoiy effectoi T cells
(CD45Ro
-
), which explains the piefeiential
depletion of these cells by Alefacept. It is given
intiamusculaily once weekly, but moie than
one-thiid of patients do not iespond foi un-
known ieasons. Repeated administiation leads
to impioved iesponse and theie may be long
peiiods of iemissions.
E]u|Izumuh is a humanized anti-CD11a mon-
oclonal antibody that blocks the inteiaction of
LFI-1 with its ligand inteicellulai adhesion
molecule 1. It is given subcutaneously once a
week and is usually highly effective, but some
patients show exaceibation of disease and theie
aie iebounds.
Tumvr necrvsIs ]uctvr (TNF) untugvnIsts
that aie effective in psoiiasis aie infiliximab,
adalimumab, and etaneicept. In[|íxíma| is a
chimeiic monoclonal antibody with a high
specificity, affinity, and avidity foi TNF- . It is
administeied as intiavenous infusion at weeks
0, 2 and 6 and is highly effective in psoiiasis
(although cuiiently only FDA appioved foi
psoiiatic aithiitis). ÀJa|ímuma| is also veiy
effective. It is a fully human iecombinant mon-
oclonal antibody that specifically taigets TNF-
. It is administeied subcutaneously eveiy othei
week and is similaily as effective as infliximab.
Eìanerte¡ì is a human iecombinant, soluble
TNF- ieceptoi that binds TNF- and neutial-
izes its activity. It is administeied as subcutane-
ous injections twice weekly and is less effective
than infliximab and adalimumab but is highly
effective in psoiiatic aithiitis.
1
Foi details and diug inteiactions, see MJ Mihatsch,
K Wolff: Consensus Confeience on Cyclospoiin A foi
Psoiiasis. Bi J Deimatol 126:621, 1992.
2
Foi details and diug inteiaction, see S Richaidson, J
Gelfand, in K Wolff et al (eds): Fíì:¡aìrít|'s Der-
maìo|ogy, ín Cenera| MeJítíne, 7th ed, New Yoik,
McGiaw-Hill, pp 2223-2236, 2008.
S£CII0N 3 |'0k|A'|' T1
AntI-Inter|eu|In(1L) 12/Inter|eu|In 2J p40
is a newei agent developed foi chionic plaque
psoiiasis and has shown piomising efficacy in
phase I tiials. It is a human IgG1 monoclonal
antibody that binds to the common p40 subunit
of human IL-12 and IL-23, pieventing its intei-
action with its ieceptoi.
All these biologicals and otheis cuiiently
developed and in clinical tiials have side effects,
and theie aie long-teim safety conceins. Also,
they aie cuiiently extiemely expensive which
limits theii use in clinical piactice. Foi doses,
wainings, and side effects see
2
and package in-
seits.
C£N£kAIII£0 FüSIüIAk FS0kIASIS
(S££ FICS. 3-13, 3-14)
These ill patients with geneialized iash should
be hospitalized and tieated in the same man-
nei as patients with extensive buins, toxic
epideimal neciolysis, oi exfoliative eiythio-
deima-in a specialized unit: isolation, fluid
ieplacement, and iepeated blood cultuies aie
necessaiy. Rapid suppiession and iesolution
of lesions is achieved by oial ietinoids (acitie-
tin, 50 mg/d). Suppoitive measuies should
include fluid intake, IV antibiotics to pievent
septicemia, caidiac suppoit, tempeiatuie con-
tiol, topical lubiicants, and antiseptic baths.
Systemic glucocoiticoids to be used only as
iescue inteivention as iapid tachyphylaxis oc-
cuis. Oial PUVA photochemotheiapy is effec-
tive, but logistics aie usually piohibitive in a
toxic patient with fevei.
ACk00£kMAIIIIS C0NIINüA hAII0F£Aü
(Figuie 3-15) Oial ietinoids as in von Zum-
busch pustulai psoiiasis; MTX, once-a-week
schedule, is the second-line choice.
FS0kIAIIC AkIhkIIIS
Should be iecognized eaily in oidei to pievent
bony destiuction. MTX, once-a-week schedule
as outlined above; infliximab oi etaneicept aie
highly effective.
T2
S E C I | 0 N 4
IChIh¥0S£S
CIASSIFICAII0N
Dominant ichthyosis vulgaiis (DIV)
X-linked iecessive ichthyosis (XLI)
Lamellai ichthyosis (LI)
Epideimolytic hypeikeiatosis (EH)
£II0I0C¥ AN0 FAIh0C£N£SIS
Individual keiatin genes may not be expiessed
oi may iesult in the foimation of abnoimal
keiatins. In DIV and XLI, foimation of thick-
ened stiatum coineum is caused by incieased
adhesiveness of the stiatum coineum cells and/
oi failuie of noimal cell sepaiation. Abnoimal
stiatum coineum foimation iesults in vaiiable
incieases in tiansepideimal watei loss. The
etiology of the most common ichthyosis, DIV,
is unknown, but theie aie mutations in the
A ç|oup o| |e|ed||+|, d|ºo|de|º c|+|+c|e||/ed
|, +u e\ceºº +ccuru|+||ou o| cu|+ueouº ºc+|e,
.+|,|uç ||or .e|, r||d +ud +º,rp|or+||c |o |||e·
|||e+|eu|uç.
A |e|+||.e|, |+|çe uur|e| o| |,peº o| |e|ed||+|,
|c|||,oºeº e\|º|, roº| +|e e\||ere|, |+|e +ud
o||eu p+|| o| ru|||o|ç+u º,ud|oreº. I|e |ou|
roº| corrou +ud |rpo||+u| |,peº +|e d|ºcuººed
|e|e p|uº + |||e| d|ºcuºº|ou o| |Wo |,peº +||ec||uç
ueW|o|uº.
'e|ec|ed |+|e, |u| |rpo||+u|, |e|ed||+|, |c|||,o·
ºeº +|e d|ºcuººed |u ||e ou||ue .e|º|ou.
Acqu||ed |c|||,oº|º c+u |e + r+u||eº|+||ou o|
º,º|er|c d|ºe+ºe, r+||çu+uc,, d|uçº, eudoc||ue
d|ºe+ºe, +u|o|rruue d|ºe+ºe, +ud n|\ +ud o||e|
|u|ec||ouº.
'uppo|| ç|oupº ºuc| +º |ouud+||ou |o| |c|||,oº|º
+ud ke|+|ed '||u I,peº (||k'I) e\|º|.
|o| +u |u·dep|| d|ºcuºº|ou o| |c|||,oºeº, ºee
| ||ec|r+u, ll ||C|o.+uu+, |u K wo||| e| +| (edº).
|·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- , 1||
ed. |eW \o||, \cC|+W·n|||, pp +0¹-+2+, 2003.
gene encoding piofilaggiin; in XLI, theie is a
steioid sulfatase deficiency. LI shows incieased
geiminative cell hypeiplasia and incieased tian-
sit iate thiough the epideimis, and theie is a
tiansglutaminase deficiency. In EH, theie aie
mutations in the genes encoding keiatins 1 oi
10; heie the distuibance of epideimal diffeien-
tiation and the expiession of abnoimal keiatin
genes iesult in vacuolization of the uppei epi-
deimal layeis, blisteiing, and hypeikeiatosis.
CIINICAI MANIF£SIAII0N
All foui types of ichthyosis tend to be woise
duiing the diy, cold wintei months and impiove
duiing the hot, humid summei. Patients living
in tiopical climates may iemain symptom-fiee
but may expeiience appeaiance oi woisening of
symptoms on moving to a tempeiate climate.
C|+|+c|e||/ed |, uºu+||, r||d çeue|+||/ed \e|oº|º
W||| ºc+||uç, roº| p|ououuced ou |oWe| |eçº, |u
ºe.e|e c+ºeº |+|çe, |eººe||+|ed ºc+|eº occu|.
n,pe|||ue+| p+|rº +ud ºo|eº.
|e|||o|||cu|+| |,pe||e|+|oº|º (|e|+|oº|º p||+||º) uºu·
+||, ou +|rº +ud |eçº.
||equeu||, +ººoc|+|ed W||| +|op,.
|C|·9 . 151.¹

|C|·¹0 . 0 30.0
00MINANI IChIh¥0SIS vüICAkIS (0Iv)
£FI0£MI0I0C¥
A¿e oI 0oset 3 to 12 months.
Sex Equal incidence in males and females.
Autosomal dominant inheiitance.
Iocìdeoce Common (1 in 250).
S£CII0N 4 |CnIn\0'E' T3
FICük£ 4-1 Ichthyosìs vu|¿arìs : chest ||ue ||º| ºc+|e|||e |,pe||e|+|oº|º o| ||e pec|o|+| +|e+. I||º |º + r||d
|o|r o| |c|||,oº|º .u|ç+||º.
FICük£ 4-2 Ichthyosìs vu|¿arìs: |e¿s
C|+,|º| |eººe||+|ed (|||e|||e), |||r|, |ouud doWu
ºc+|eº. I|e º|r||+|||, |o ||º| º||u o| ||e º||u o| +u
+rp||||+u |º qu||e o|.|ouº. |o|e ºp+||uç o|
pop|||e+| |oºº+e. I||º |º + ro|e ºe.e|e |o|r o|
|c|||,oº|º .u|ç+||º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' T4
FAIh0C£N£SIS
Etiology unknown. Theie is ieduced oi absent
filaggiin. Epideimis piolifeiates noimally, but
keiatin is ietained with a iesultant thickened
stiatum coineum.
CIINICAI MANIF£SIAII0N
Veiy commonly associated with atopy. Xeiosis
and piuiitus woise in wintei months. Cosmetic
concein to many patients, paiticulaily when
hypeikeiatosis is seveie.
Skìo Iesìoos Xeiosis (diy skin) with fine,
powdeiy scaling but also laigei, fiimly adheient
tacked-down scales in a fish-scale pattein (Figs.
4-1 and 4-2). Diffuse geneial involvement,
accentuated on the shins, aims, and back but
also on the buttocks and lateial thighs; axillae
and the antecubital and popliteal fossae spaied
(Fig. 4-2; Image 4-1); face usually also spaied
but cheeks and foiehead may be involved.
Keraìosís ¡í|arís is peiifolliculai hypeikeiatosis
with little, spiny hypeikeiatotic folliculai
papules of noimal skin coloi, eithei giouped oi
disseminated, mostly on the extensoi suifaces
of the extiemities (Fig. 4-3); in childhood, also
on cheeks. Hands and feet usually spaied, but
palmoplantai maikings aie moie accentuated
(hypeilineai).
Assocìated 0ìseases Moie than 50% of indi-
viduals with DIV also have atopic deimatitis;
iaiely, keiatopathy can occui.
0IFF£k£NIIAI 0IACN0SIS
Xerosìs[hyperkeratosìs Xeiosis; acquiied ich-
thyoses, all othei foims of ichthyosis.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Compact hypeikeiatosis;
ieduced oi absent gianulai layei; geimina-
tive layei flattened. Election micioscopy: small,
pooily foimed keiatohyalin gianules.
0IACN0SIS
Usually by clinical findings; abnoimal keiato-
hyalin gianules in election micioscopy.
C0ükS£ AN0 Fk0CN0SIS
Impiovement in the summei, in humid cli-
mates, and in adulthood. Keiatosis pilaiis oc-
cuiiing on the cheeks duiing childhood usually
impioves duiing adulthood.
MANAC£M£NI
hydratìoo oI Stratum Coroeum Pliability of
stiatum coineum is a function of its watei
content. Hydiation best accomplished by im-
meision in a bath followed by the application of
petiolatum. Uiea-containing cieams bind watei
in the stiatum coineum.
kerato|ytìc A¿eots Piopylene glycol-glyceiin-
lactic acid mixtuies. Piopylene glycol (44-60%
in watei); 6% salicylic acid in piopylene glycol
and alcohol, which is used undei plastic oc-
clusion (bewaie of hypeisalicism). -Hydioxy
acids (lactic acid oi glycolic acid) contiol scal-
ing. Uiea-containing piepaiations (2-10%) aie
effective.
Systemìc ketìooìds Isotietinoin and acitietin
aie veiy effective, but caieful monitoiing foi
toxicity is iequiied. Only seveie cases may
iequiie inteimittent theiapy.
IMAC£ 4-1 0ìstrìbutìoo oI ìchthyosìs vu|¿arìs
|o|º |ud|c+|e |e|+|oº|º p||+||º
S£CII0N 4 |CnIn\0'E' T5
0ccu|º |u r+|eº.
0uºe| ºoou +||e| |||||.
||or|ueu|, d|||, ||oWu ºc+|eº ou ||e uec|,
e\||er|||eº, ||uu|, +ud |u||oc|º.
|u.o|.ereu| o| ||e\u|+| |eç|ouº.
A|ºeuce o| p+|r +ud ºo|e |u.o|.ereu|.
Co|ue+| op+c|||eº |u 50° o| +du|| r+|eº.
X-IINk£0 IChIh¥0SIS (XII) |C|·9 . 151.¹

|C|·¹0 . 0 30.¹
£FI0£MI0I0C¥
A¿e oI 0oset Biith oi infancy. Males.
Iocìdeoce 1:2000 to 1:6000.
£II0I0C¥ AN0 FAIh0C£N£SIS
X-linked iecessive; gene locus Xp22.32.
Ceoetìc 0eIect Steioid sulfatase deficiency-
abnoimal cholesteiol metabolism, accumulation
of cholesteiol sulfate; is associated with failuie to
shed senescent keiatinocytes noimally, iesulting
clinically in ietention hypeikeiatosis associated
with noimal epideimal piolifeiation.
CIINICAI MANIF£SIAII0N
Onset of skin abnoimality at 2-6 weeks of age;
coineal opacities develop duiing the second to
thiid week. Usually asymptomatic; may also be
piesent in female caiiieis of XLI. Discomfoit
due to xeiosis. Cosmetic disfiguiement due to
the diity biown scales.
Skìo Iesìoos Laige adheient scales that
appeai biown oi diity (Fig. 4-4); most
pionounced on posteiioi neck, extensoi aims,
antecubital and popliteal fossae, and tiunk.
Absence of palm/sole and face involvement
(Image 4-2).
FICük£ 4-3 Ichthyosìs vu|¿arìs. keratosìs pì|arìs: arm 'r+||, |o|||cu|+|, |o|u, ºp|ueº occu| +º + r+u|·
|eº|+||ou o| r||d |c|||,oº|º .u|ç+||º, +||º|uç roº||, ou ||e º|ou|de|º, uppe| +|rº, +ud |||ç|º. |eºqu+r+||ou o|
||e uou|o|||cu|+| º||u |eºu||º |u |,pore|+uo||c (|eºº p|çreu|ed) ºpo|º º|r||+| |o p||,||+º|º +||+ (corp+|e W|||
||ç. ¹!·¹o).
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' T6
£ye Iesìoos Comma-shaped stiomal coineal
opacities in 50% of adult males, asymptomatic.
Piesent in some female caiiieis.
Ceoìtourìoary Aboorma|ìty Ciyptoichidism
in 20% of individuals.
0IFF£k£NIIAI 0IACN0SIS
All foims of ichthyosis, syndiomic ichthyoses.
IA80kAI0k¥ £XAMINAII0NS
Chemìstry Cholesteiol sulfate level elevated.
Incieased mobility of -lipopioteins in electio-
phoiesis. Steioid sulfatase decieased oi absent.
0ermatopatho|o¿y Hypeikeiatosis; gianulai
layei piesent, sometimes hypeigianulosis.
0IACN0SIS
By family histoiy and clinical findings.
Freoata| 0ìa¿oosìs Via amniocentesis and
choiionic villus sampling; steioid sulfatase assay
detects enzyme deficiency.
C0ükS£ AN0 Fk0CN0SIS
No impiovement with age. Usually woise in
tempeiate climates and in wintei season. With
placental sulfatase deficiency, failuie of laboi to
begin oi piogiess in mothei caiiying affected
fetus.
MANAC£M£NI
Iopìca| Iherapy
hydratìoo oI Stratum Coroeum Pliability of
stiatum coineum is a function of its watei
content. Hydiation best accomplished by im-
meision in a bath followed by the application of
petiolatum. Uiea-containing cieams bind watei
in the stiatum coineum.
kerato|ytìc A¿eots Piopylene glycol-glyceiin-
lactic acid mixtuies. Piopylene glycol (44-60%
in watei); 6% salicylic acid in piopylene glycol
and alcohol, which is used undei plastic occlu-
sion. -Hydioxy acids (lactic acid oi glycolic
acid) (bewaie of hypeisalicism) contiol scal-
ing. Uiea-containing piepaiations (2-10%) aie
effective.
Systemìc ketìooìds Isotietinoin and acitietin
aie veiy effective, but caieful monitoiing foi
toxicity is iequiied. Only seveie cases may
iequiie inteimittent theiapy.
Systemìc Ireatmeot
Àtíìreìín , 0.5-1 mg/kg oially until maiked im-
piovement, then tapei dose to maintenance
level. Continuous laboiatoiy monitoiing and,
in long-teim iegimens, x-iays foi calcifications
and diffuse idiopathic skeletal hypeiostosis
(DISH) syndiome mandatoiy.
IMAC£ 4-2 0ìstrìbutìoo oI
X-|ìoked ìchthyosìs.
S£CII0N 4 |CnIn\0'E' TT
FICük£ 4-4 X-|ìoked ìchthyosìs: truok, buttocks aod arms |+|| |,pe||e|+|oº|º W||| |eººe||+|ed ºc+|eº
ç|.eº + d|||, +ppe+|+uce |u |||º ¹2·,e+|·o|d |o, o| A|||c+u e||u|c||,.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' T8
0||eu p|eºeu|º +| ||||| +º co||od|ou |+|, (ºee
p+çe 13)
Co||od|ou·|||e rer||+ue ºoou º|ed W||| ºu|ºe·
queu| |+|çe, co+|ºe ºc+|eº |u.o|.|uç eu|||e |od,.
||e\u|+| +|e+º |u.o|.ed.
|+|rop|+u|+| |u.o|.ereu| .+||eº.
E\||op|ur, ec|+||ur, +|opec|+ r+, occu|.
ne+| |u|o|e|+uce.
IAM£IIAk IChIh¥0SIS (II) |C|·9 . 151.¹

|C|·¹0 . 0 30.2
£FI0£MI0I0C¥
A¿e oI 0oset At biith, usually as collodion
baby.
Sex Piesents equally in both sexes.
Iocìdeoce 1:300,000.
£II0I0C¥ AN0 FAIh0C£N£SIS
Mode oI Ioherìtaoce Autosomal iecessive;
gene locus vaiies. In one subset theie is muta-
tion in the gene encoding tiansglutaminase 1,
an enzyme that catalyzes the cioss-linking of
pioteins duiing the foimation of coinified en-
velopes of coineocytes. In anothei, a mutation
in the gene encoding ATP-binding cassette, sub-
family A, membei 12-contiolling membiane
tianspoit/lipid metabolism. In a thiid subset,
theie is a mutation in the gene encoding aia-
chidonate lipoxygenase, contiolling hydiopei-
oxidase isomeiase .
1
CIINICAI MANIF£SIAII0N
Heat intoleiance, usually duiing exeicise and
hot weathei, because of inability to sweat. Watei
loss (excess)/dehydiation due to fissuiing of
stiatum coineum. Incieased nutiitional ie-
quiiements foi young childien due to iapid
giowth and shedding of stiatum coineum.
Painful palmai/plantai fissuies.
Skìo Iesìoos Newhvrn Collodion baby,
encased in a tianslucent collodion-like mem-
biane (see Fig. 4-8); shed in a few weeks.
Ectiopion; eclabion. Geneialized eiythiodeima.
1
Foi details on genes identified in autosomal ieces-
sive ichthyoses, see P Fleckman, JJ DiGiovanna,
in K Wolff et al: Fíì:¡aìrít|'s Dermaìo|ogy ín Cen-
era| MeJítíne , 7th ed. New Yoik, McGiaw-Hill,
pp 401-424, 2008.
1
Foi details on genes identified in autosomal ieces-
sive ichthyoses, see P Fleckman, JJ DiGiovanna,
in K Wolff et al: Fíì:¡aìrít|'s Dermaìo|ogy ín Cen-
era| MeJítíne , 7th ed. New Yoik, McGiaw-Hill,
pp 401-424, 2008.
ChI|d/Adu|t Laige paichment-like hypeikeia-
tosis (Fig. 4-5) ovei entiie body; fiactuiing of
the hypeikeiatotic plate iesults in a tessellated
(tilelike) pattein (Fig. 4-6). Scales aie laige and
veiy thick and biown, ovei most of the body
(Fig. 4-6), accentuated on lowei extiemities,
and involving the flexuial aieas. Hypeikeiatosis
aiound joints may be veiiucous. . Hands/feet:
keiatodeima; accentuation of palmai/plantai
cieases (Image 4-3) but may vaiy. Eiythio-
deima may develop.
haìr Bound down by scales; fiequent infec-
tions may iesult in scaiiing alopecia (Fig. 4-5).
Naì|s Dystiophy secondaiy to nail fold in-
flammation.
Mucous Membraoes Usually spaied.
£ye Iesìoos Ectiopion (Fig. 4-5).
Iìps Eclabium.
0IFF£k£NIIAI 0IACN0SIS
X-linked ichthyosis, epideimolytic hypeikeia-
tosis, congenital ichthyosifoim eiythiodeima,
syndiomic ichthyoses.
IA80kAI0k¥ £XAMINAII0NS
Cu|ture Rule out secondaiy infection and
sepsis, especially in newboins.
0ermatopatho|o¿y Hypeikeiatosis; gianulai
layei piesent; acanthosis. Epideimal tiansgluta-
minase decieased in tiansglutaminase-deficient
subtype.
C0ükS£ AN0 Fk0CN0SIS
Collodion membiane piesent at biith is shed
within fiist few days to weeks (see Fig. 4-8). New-
boins aie at iisk foi hypeinatiemic dehydiation,
secondaiy infection, and sepsis. Disoidei pei-
sists thioughout life. No impiovement with age.
S£CII0N 4 |CnIn\0'E' T9
IMAC£ 4-3 0ìstrìbutìoo oI
|ame||ar ìchthyosìs.
FICük£ 4-5 Iame||ar
ìchthyosìs |+|c|reu|·|||e
|,pe||e|+|oº|º ç|.eº ||e
|rp|eºº|ou o| ||e º||u |e|uç
|oo ||ç|| ou ||e |+ce o| |||º
o·,e+|·o|d A|+| |o,. I|e|e
|º p|ououuced ec||op|ur
+ud |eç|uu|uç +|opec|+.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 80
Obstiuction of ecciine sweat glands with iesult-
ant impaiiment of sweating.
MANAC£M£NI
Newboro Caie in neonatal intensive caie unit.
High-humidity chambei. Emolliation. Monitoi
electiolytes, fluids. Follow foi signs of local oi
systemic infection.
Chì|d[Adu|t Emv||Ients Hydiated petiola-
tum.
Kerutv|ytIcs As in DIV and XLI.
0verheatìo¿ Paients and affected individuals
should be instiucted about oveiheating and
heat piostiation that can follow exeicise,
high enviionmental tempeiatuies, and fevei.
Repeated application of watei to skin can some-
what ieplace function of sweating, cooling the
body.
ketìooìds Acitietin and, to a lessei degiee,
isotietinoin (0.5-1 mg/kg) aie effective. Monitoi
continuously foi seium tiiglyceiides, tiansami-
nases, and bony toxicities if given ovei pio-
longed peiiod of time. Teiatogenicity iequiies
effective contiaception.
FICük£ 4-6 Iame||ar ìchthyosìs: Shou|der Ieººe|+|ed (|||e|||e) |,pe||e|+|oº|º ç|.eº ||e +ppe+|+uce o|
|ep||||+u ºc+|eº ou ||e º|ou|de| +ud |+c|. I|e eu|||e |od, W+º |u.o|.ed +ud ||e|e W+º ec||op|ur.
S£CII0N 4 |CnIn\0'E' 81
||eºeu|º +| o| º|o|||, +||e| ||||| W||| |||º|e||uç.
w||| ||re º||u |ecoreº |e|+|o||c, .e||ucouº.
'|edd|uç o| |,pe||e|+|o||c r+ººeº |eºu||º |u
c||curºc|||ed |º|+udº o| uo|r+|·+ppe+||uç º||u.
|u.o|.ereu| o| ||e\u|+| +|e+º.
|+|r+| +ud p|+u|+| |u.o|.ereu|.
'ecoud+|, p,oçeu|c |u|ec||ouº.
£FI0£kM0I¥IIC h¥F£kk£kAI0SIS (£h)
|C|·9 . 151.¹

|C|·¹0 . 0 30.3
£FI0£MI0I0C¥
A¿e oI 0oset Biith oi shoitly theieaftei.
Sex Equal incidence in males and females.
Iocìdeoce Veiy iaie.
£II0I0C¥ AN0 FAIh0C£N£SIS
Mode oI Ioherìtaoce Autosomal dominant.
Mutations of genes that encode the epideimal
diffeientiation keiatins, keiatin 1 and 10.
2
Stiuc-
tuial piotein abnoimality keiatin inteimedi-
ate filament dysfunction epideimal fiagility.
CIINICAI MANIF£SIAII0N
Blisteiing may iecui peiiodically, leading to
denuded aieas, secondaiy infection, and sepsis.
Hypeikeiatotic lesions become veiiucous, pai-
ticulaily in the flexuial aieas, and aie associated
with an unpleasant odoi.
Skìo Iesìoos Blisteiing at biith oi shoitly
theieaftei. Geneialized oi localized. Denuded
aieas heal with noimal-appeaiing skin. With
time, the skin becomes keiatotic and veiiucous
(Fig. 4-7), paiticulaily in the flexuial aieas,
knees, and elbows. Hypeikeiatotic scales adheie
to undeilying skin, often in a mountain iange-
like appeaiance; may be quite daik in coloi and
associated with an unpleasant odoi (like iancid
buttei). Recuiient blisteis in hypeikeiatotic aieas
(Fig. 4-7) and also shedding of hypeikeiatotic
masses iesult in ciicumsciibed aieas of skin that
aie ielatively noimal in appeaiance. A valuable
diagnostic sign. Secondaiy pyogenic infections,
especially impetigo.
Geneialized distiibution with piominent in-
volvement of flexuial aieas (Image 4-4). Palmai
and plantai involvement (hypeikeiatosis).
(Noìe : A vaiiant of epideimolytic hypeikeiato-
sis is localized to palms and soles and is geneti-
cally distinct fiom the geneialized foim.)
2
Foi othei subtypes and genes involved, see P Fleck-
man, JJ DiGiovanna, in K Wolff et al (eds): Fíì:¡aì-
rít|'s Dermaìo|ogy ín Cenera| MeJítíne , 7th ed. New
Yoik, McGiaw-Hill, pp 401-424, 2008.
2
Foi othei subtypes and genes involved, see P Fleck-
man, JJ DiGiovanna, in K Wolff et al (eds): Fíì:¡aì-
rít|'s Dermaìo|ogy ín Cenera| MeJítíne , 7th ed. New
Yoik, McGiaw-Hill, pp 401-424, 2008.
haìr aod Naì|s Haii noimal, but involvement
of the nails may pioduce abnoimal nail plates.
Mucous Membraoes Spaied.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Giant, coaise keiatohyalin
gianules and vacuolization of the gianulai layei,
iesulting in cell lysis and subcoineal multilocu-
lated blisteis. Papillomatosis, acanthosis, and
hypeikeiatosis.
C0ükS£ AN0 Fk0CN0SIS
Blistei foimation and massive hypeikeiatosis
aie pione to bacteiial supeiinfection, which is
piobably also iesponsible foi the unpleasant
odoi. Palmai involvement can adveisely affect
manual dexteiity.
MANAC£M£NI
Topical application of -hydioxy acids. Antimi-
ciobial theiapy. Systemic ietinoids (isotietinoin
and acitietin) may tiansiently lead to a wois-
ening of the condition because of incieased
blistei foimation but latei impiove the skin
diamatically owing to a ielative noimalization
of epideimal diffeientiation. Piedisposes to im-
petigo. Deteimine dose caiefully, and monitoi
foi side effects.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 82
FICük£ 4-T £pìdermo|ytìc hyperkeratosìs: arms aod haods \ouu|+|u |+uçe|||e |,pe||e|+|oº|º o| ||e
do|ºur o| |+udº W||| |||º|e||uç ||+| |eºu||º |u e|oº|ouº +ud º|edd|uç o| |+|çe º|ee|º o| |e|+||u.
IMAC£ 4-4 0ìstrìbutìoo oI
epìdermo|ytìc hyperkeratosìs.
S£CII0N 4 |CnIn\0'E' 83
IChIh¥0SIS IN Ih£ N£W80kN
MANAC£M£NI
Newboins should be kept in an incubatoi in
which the aii is satuiated with watei. Caie-
ful monitoiing of tempeiatuie and paienteial
fluids and nutiient ieplacement may be neces-
saiy foi some time. Infection of the skin and
lungs is an impoitant pioblem, and aggiessive
antibiotic theiapy may be indicated.
Euc+ºereu| o| eu|||e |+|, |u + ||+uºp+|eu| p+|c|·
reu|·|||e rer||+ue (||ç. +·3A) |rp+||º |eºp||+·
||ou +ud ºuc||uç.
B|e+||uç +ud º|edd|uç o| ||e co||od|ou rer·
||+ue |u|||+||, |e+dº |o d||||cu|||eº |u ||e|ro|eçu|+·
||ou +ud |uc|e+ºed ||º| o| |u|ec||ou.
'||u |º |||ç|| |ed +ud ro|º|. A||e| |e+||uç, º||u
+ppe+|º uo|r+| |o| ºore ||re uu||| º|çuº o| |c|·
||,oº|º de.e|op.
Co||od|ou |+|, r+, |e ||e |u|||+| p|eºeu|+||ou o|
|+re||+| |c|||,oº|º o| ºore |eºº corrou |o|rº
o| |c|||,oº|º uo| d|ºcuººed |e|e.
Co||od|ou |+|, +|ºo r+, |e + coud|||ou W||c|,
+||e| ||e co||od|ou rer||+ue |º º|ed +ud ||e
|eºu||+u| e|,||er+ |+º c|e+|ed, W||| p|oç|eºº |o
uo|r+| º||u |o| ||e |eº| o| ||e c|||d'º |||e (||ç.
+·3B).
C0II00I0N 8A8¥ |C|·9 . 151.¹

|C|·¹0 . 0 30.2
FICük£ 4-8 Ichthyosìs ìo the
oewboro
 'Co||od|ou |+|,¨ º|o|||, +||e|
||||| W||| + p+|c|reu|·|||e rer·
||+ue co.e||uç ||e eu|||e º||u.I|e
rer||+ue |+º |up|u|ed +ud |º |e|uç
º|ed |e+.|uç oo/|uç, |+W·|oo||uç º||u.
 A| 3 rou||º o| +çe, ||e º+re
|u|+u| |º + |e+u|||u| |+|, W||| r|u|·
r+| |eº|du+| ºc+|e +ud e|,||er+.


FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 84
I|eºe +|e + uur|e| o| |+|e º,ud|or|c |c|||,oºeº
W|e|e |c|||,o||c º||u c|+uçeº +|e +ººoc|+|ed W|||
re|+|o||c +ud/o| |uuc||ou+| +ud º||uc|u|+| +|uo|·
r+||||eº.
|o| -·,||·¤!-·¤c .c··c|·|·º |-·c|·|·º··:|||,¤º·º
c¤! !-c|¤-ºº (|||) º,¤!·¤¤- C|·|! º,¤!·¤¤-
+ud |-||-·|¤¤ º,¤!·¤¤- ºee |c|||,oº|º p|c|u|e
ç+||e|, |u ||e ou||ue .e|º|ou.
|o| o||e|º ºee | ||ec|r+u, ll ||C|o.+uu+, |u
K wo||| e| +|. |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤·
-·c| !-!·:·¤-, 1 || ed. |eW \o||, \cC|+W·n|||, pp
+0¹-+2+, 2003.
S¥N0k0MIC IChIh¥0S£S |C|·9 . 151.¹

|C|·¹0 . 0 30.9
|e.e|opreu| |u +du|||ood.
Aººoc|+|ed W||| r+||çu+uc|eº (nodç||u d|ºe+ºe
|u| +|ºo uou·nodç||u |,rp|or+º +ud o||e|
r+||çu+uc|eº).
Aººoc|+|ed W||| A||'.
Aººoc|+|ed W||| º+|co|doº|º.
Aººoc|+|ed W||| º,º|er|c |upuº e|,||er+|oºuº,
de|r+|or,oº|||º, r|\ed couuec||.e ||ººue d|ºe+ºe,
eoº|uop||||c |+ºc||||º.
Aººoc|+|ed W||| ç|+||·.e|ºuº·|oº| d|ºe+ºe.
Aººoc|+|ed W||| d|uçº (u|co||u|c +c|d, |||p+u+|o|,
|u|,|op|euo|, d|\,|+/|ue, u+|o\|d|ue.
0ccu|º |u K+.+ d||u|e|º. |c.c !-·¤¤¤c||, .
ACÇüIk£0 IChIh¥0S£S |C|·9 . 10¹.¹

|C|·¹0 . | 35.0
|+|rop|+u|+| |e|+|ode|r+º (||K) +|e + |+|e +ud
d|.e|ºe ç|oup o| |e|+||u|/+||ou d|ºo|de|º.
||K r+, |e |ouud +|oue o| coucor||+u| W|||
(|e|+|ed) |eº|ouº e|ºeW|e|e ou ||e |od, o| r+,
|e p+|| o| corp|e\ º,ud|oreº.
C||u|c+| c|+ºº|||c+||ou d|º||uçu|º|eº |e|Weeu d||·
|uºe, |oc+| (º|||+|e), +ud puuc|+|e ||K.
',rp|orº |uc|ude p+|u, +ººoc|+|ed W||| r+uu+|
|+|o| +ud W+|||uç, +ud ºecoud+|, |u|ec||ouº.
I|e çeue||c |+º|º o| roº| |KK |u.o|.eº ru|+||ouº
|u |e|+||u çeueº o| çeueº eucod|uç couue\|u +ud
deºroºor+| p|o|e|uº.
I|e+|reu| couº|º| o| |e|+|o|,||cº +ud º,º|er|c
|e||uo|dº.
INh£kII£0 k£kAI00£kMAS 0F FAIMS AN0 S0I£S
CIASSIFICAII0N
Theie exist moie than twenty diffei-
ent PKK eithei confined to palms and soles
(simple) oi in combination with lesions else-
wheie (complex) oi as pait of a multioi-
gan syndiome (syndiomic). The undeilying
gene defects foi almost eveiyone of these aie
n+||equ|u |e|uº |º +u e\||ere|, |+|e coud|||ou |u
W||c| ||e c|||d |º |o|u W||| .e|, |||c| p|+|eº o|
º||+|ur co|ueur ºep+|+|ed |, deep c|+c|º +ud
||ººu|eº.
Ec|+||ur, ec||op|ou, +ud +|ºeuce o| o| |ud|reu·
|+|, e+|º |eºu|| |u + ç|o|eºque +ppe+|+uce.
I|eºe |+||eº uºu+||, d|e º|o|||, +||e| |||||, |u|
||e|e +|e |epo||º o| ºu|.|.+| |o| Wee|º |o ºe.e|+|
rou||º.
I||º coud|||ou |º d|||e|eu| ||or co||od|ou |+|,
+ud ||e o||e| |o|rº o| |c|||,oº|º, W||| +u uuuºu+|
||||ouº p|o|e|u W||||u ||e ep|de|r|º.
hAkI£ÇüIN F£IüS |C|·9 . 151.¹

|C|·¹0 . 0 30.+
S£CII0N 4 |CnIn\0'E' 85
unknown.
3
In this book only thiee simple PKK
phenotypes will be discussed:
Diffuse PKK
Punctate PKK
Focal PKK
0IFFüS£ FAIM0FIANIAk
k£kAI00£kMA
Two main types exist. Epideimolytic and
nonepideimolytic. Nonepideimolytic PKK is
autosomal dominant, piesenting in infancy; it
3
Foi additional discussion, including genetics and
pathogenesis, the ieadei is ieffeied to DP Kelsell, IM
Leigh, in K Wolff et al: Fíì:¡aìrít|'s Dermaìo|ogy ín
Cenera| MeJítíne , 7th ed. New Yoik, McGiaw-Hill,
pp 424-431, 2008.
3
Foi additional discussion, including genetics and
pathogenesis, the ieadei is ieffeied to DP Kelsell, IM
Leigh, in K Wolff et al: Fíì:¡aìrít|'s Dermaìo|ogy ín
Cenera| MeJítíne , 7th ed. New Yoik, McGiaw-Hill,
pp 424-431, 2008.
consists of symmetiic well-demaicated diffuse
waxy thickening of the stiatum coineum of
palms and soles (Fig. 4-9). Spiead to doisum of
hands and wiists occuis. Epideimolytic diffuse
PKK is also autosomal dominant; the keiato-
deima is also well-defined, diffuse, and sym-
metiic but is not waxy but has fine fissuiing on
the suiface.
Symptoms, if at all, consist of pain with
manual woik and walking.
FüNCIAI£ FAIM0FIANIAk
k£kAI00£kMA
An autosomal dominant PKK aiising in adoles-
cence and consisting of multiple punctate keia-
toses symmetiically on palms and soles (Fig.
4-10). Lesions may iesemble palmai/plantai
waits and get woise by physical tiauma. Tendei-
ness and pain.
FICük£ 4-9 F|aotar keratoderma, dìIIuse type \e||oW W+\, d|||uºe |,pe||e|+|oº|º ou |o|| ºo|eº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 86
F0CAI FAIM0FIANIAk
k£kAI00£kMA
Mostly stiiate in appeaiance, autosomal domi-
nant. Lineai hypeikeiatotic calluses usually ex-
tending fiom the palm to the tips of the fingeis
(Fig. 4-11). Aiises in childhood and gets woise
with manual laboi.
IA80kAI0k¥ £XAMINAII0N
Histopathology nondiagnostic in nonepidei-
molytic PKK. Hypeikeiatosis, acanthosis, papil-
lomatosis. In epideimolytic PKK, epideimolytic
hypeikeiatosis.
0IFF£k£NIIAI 0IACN0SIS
Hypeikeiatotic chionic iiiitant deimatitis, pso-
iiasis, calluses, palmai/plantai waits.
FICük£ 4-10 Fuoctate p|aotar keratoderma \u|||p|e, d|ºc|e|e d|op|||e |e|+|oºeº |eºer|||uç p|+u|+|
W+||º. |eº|ouº |+d |eeu p|eºeu| º|uce |+|e c|||d|ood +ud |+.e |ecore Wo|ºe, p+|||cu|+||, |u ||e p|eººu|e +|e+º.
S£CII0N 4 |CnIn\0'E' 8T
C0ükS£ AN0 Fk0CN0SIS
Does not impiove with age, life-long com-
panion. Gets woise with physical tiauma (man-
ual laboi, walking); complications aie bacteiial
and fungal infections.
MANAC£M£NI
TvpIcu| Physical examination débiidement
(chiiopody) ieduces keiatotic masses. Topical
keiatolytic agents: 10-20% salicylic acid
ointment, coin plasteis (20-40% salicylic acid).
40-60% piopylene glycol undei oveinight
occlusion.
SystemIc Acitietin oi, in women of childbeai-
ing age, isotietinoin, 0.5 mg/kg body weight
veiy beneficial, but shedding of hypeikeiatosis
is often associated with incieased sensitivity,
which may inteifeie with manual woik and
walking. Blisteiing may occui in epideimolytic
PKK. Bewaie of teiatogenicity and long-teim
complications.
FICük£ 4-11 Strìate (Ioca|) pa|mar keratoderma I|e|e +|e ||ue+| .e||ucouº |,pe||e|+|oºeº e\|eud|uç
||or ||e p+|r ou|o ||e ||uçe|º. \+uu+| Wo|| +çç|+.+|eº ||eºe |eº|ouº, W||c| c+u |ecore ||ººu|ed +ud p+|u|u|.
88
S E C I | 0 N 5
MISC£IIAN£0üS
£FI0£kMAI 0IS0k0£kS
CIASSIFICAII0N
Iype 1: heredìtary 8eoì¿o AN No associated
endociine disoidei.
Iype 2: 8eoì¿o AN Endociine disoideis associ-
ated with insulin iesistance: insulin-iesistant
type II diabetes mellitus, hypeiandiogenic
states, aciomegaly/gigantism, Cushing disease,
hypogonadal syndiomes with insulin iesistance,
Addison disease, hypothyioidism.
Iype 3: Fseudo-AN Associated with obesity;
moie common in patients with daikei pigmen-
tation. Common in metabolic syndiome. Obes-
ity pioduces insulin iesistance.
Iype 4: 0ru¿-ìoduced AN Nicotinic acid in
high dosage, stilbestiol in young males, gluco-
coiticoid theiapy, diethylstilbestiol/oial contia-
ceptive, giowth hoimone theiapy.
Iype 5: Ma|ì¿oaot AN Paianeoplastic, usually ad-
enocaicinoma of gastiointestinal oi genitouiinaiy
tiact; less commonly, lymphoma (see Section 18).
£FI0£MI0I0C¥
A¿e oI 0oset Type 1: duiing childhood oi
pubeity; othei types dependent on associated
conditions.
£II0I0C¥ AN0 FAIh0C£N£SIS
Dependent on associated disoidei. In a subset
of women with hypeiandiogenism and insulin
Aº,rre|||c .e|.e|, |||c|eu|uç +ud |,pe|p|ç·
reu|+||ou o| ||e º||u, c||e||, ou ||e uec|, +\|||+,
ç|o|uº, +ud o||e| |od, |o|dº.
\+, |e |,pe||e|+|o||c +ud +ººoc|+|ed W||| º||u
|+çº.
A cu|+ueouº r+||e| |e|+|ed |o |e|ed||,, o|eº||,,
eudoc||ue d|ºo|de|º (p+|||cu|+||, d|+|e|eº), d|uç
+dr|u|º||+||ou, +ud r+||çu+uc,.
|uº|d|ouº ouºe|, |u r+||çu+uc,, |+p|d.
ACANIh0SIS NICkICANS (AN)
|C|·9 . 10¹.2

|C|·¹0 . | 3!     
intoleiance and AN, loss-of-function muta-
tion in the insulin ieceptoi oi anti-insulin
ieceptoi antibodies can be found (types A
and B). It is postulated that excess giowth
factoi stimulation in the skin leads to pio-
lifeiation of keiatinocytes and fibioblasts. In
hypeiinsulinemia AN, excess insulin binding
to insulin-like giowth factoi 1 ieceptoi and
fibioblast giowth factoi ieceptoi has also
been implicated. In malignancy-associated
AN, tiansfoiming giowth factoi ieleased
fiom tumoi cells may stimulate keiatinocyte
piolifeiation via epideimal giowth factoi ie-
ceptois.
CIINICAI MANIF£SIAII0N
Insidious onset; fiist visible change is daikening
of pigmentation.
Skìo Iesìoos All types of AN: Daikening
of pigmentation, skin appeais diity (Fig.
5-1). As skin thickens, appeais velvety; skin
lines accentuated; suiface becomes iugose,
mammillated. Type 3: velvety patch on innei,
uppei thigh at site of chafing; often has
many skin tags in body folds and neck. Type
5: hypeikeiatosis and hypeipigmentation moie
pionounced (see Fig. 18-16). Hypeikeiatosis
of palms/soles, with accentuation of papillaiy
maikings: °Tiipe hands" (see Fig. 18-18),
involvement of oial mucosa and veimilion
boidei of lips (see Fig. 18-17).
S£CII0N 5 \|'CE||A|E0u' E|||Ek\A| ||'0k|Ek' 89
DIstrIhutIvn Most commonly, axillae; (Fig. 5-1),
neck (back, sides) also, gioins, anogenitalia,
antecubital fossae, knuckles, submammaiy, um-
bilicus.
Mucous Membraoes Oial mucosa: velvety
textuie with delicate fuiiows. Type 5: Mu-
cous membianes and mucocutaneous junc-
tions commonly involved; waity papillomatous
thickenings peiioially (see Fig. 18-17).
Ceoera| £xamìoatìoo
Examine foi undeilying endociine disoideis
in oveiweight to moibidly obese peisons and
malignancy.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
C|ìoìca| Fìodìo¿s Daik thickened flexuial
skin: Confluent and ieticulated papillomato-
sis (Gougeiot-Caiteaud syndiome), pityiiasis
veisicoloi, X-linked ichthyosis, ietention hypei-
keiatosis, nicotinic acid ingestion.
IA80kAI0k¥ £XAMINAII0NS
Chemìstry Rule out diabetes mellitus; meta-
bolic syndiome
0ermatopatho|o¿y Papillomatosis, hypeikeia-
tosis; epideimis thiown into iiiegulai folds,
showing vaiious degiees of acanthosis.
Ima¿ìo¿ aod £odoscopy Rule out associated
malignancy.
C0ükS£ AN0 Fk0CN0SIS
Type 1: Accentuated at pubeity and, at times,
iegiesses when oldei. Type 2: Depends on un-
deilying distuibance. Type 3: May iegiess aftei
significant weight loss. Type 4: Resolves when
causative diug is discontinued. Type 5: AN may
piecede othei symptoms of malignancy by 5
yeais; iemoval of malignancy may be followed
by iegiession of AN.
MANAC£M£NI
Symptomatic. Tieat associated disoidei. Topical
keiatolytic and/oi topical oi systemic ietinoids
may impiove AN.
FICük£ 5-1 Acaothosìs oì¿rìcaos \e|.e|,,
d+||·||oWu |o ç|+, |||c|eu|uç o| ||e º||u o| ||e
+|rp|| W||| p|or|ueu| º||u |o|dº +ud |e+||e|ed edçeº
|u + !0·,e+|·o|d o|eºe Wor+u ||or ||e \|dd|e E+º|.
I|e|e We|e º|r||+| c|+uçeº ou ||e uec|, ||e +u|ecu·
|||+| |oºº+e, +ud ou ||e |uuc||eº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 90
A |+|e +u|oºor+| dor|u+u| |u|e|||ed d|ºe+ºe W|||
|+|e ouºe|.
\u|||p|e d|ºc|e|e ºc+||uç, c|uº|ed +ud p|u||||c
p+pu|eº r+|u|, |u ºe|o|||e|c +ud ||e\u|+| +|e+º.
\+|odo|ouº +ud d|º||çu||uç, +|ºo |u.o|.|uç u+||º
+ud rucouº rer||+ueº.
||c||uç +ud/o| p+|u|u|.
n|º|o|oç|c+||, c|+|+c|e||/ed |, ºup|+|+º+| +c+u·
||o|,º|º +ud d,º|e|+|oº|º.
C+uºed |, |oºº·o|·|uuc||ou ru|+||ou |u ||e 1í|212
çeue.
',uou,r. |+||e|·w|||e d|ºe+ºe, |e|+|oº|º |o|||cu·
|+||º.
0AkI£k 0IS£AS£ (00) |C|·9 . 10¹.¹

|C|·¹0 . | 31 
£FI0£MI0I0C¥ AN0 £II0I0C¥
Raie.
A¿e oI 0oset Usually in the fiist oi second
decade, males and females equally affected.
Ceoetìcs Autosomal dominant tiait, new
mutations common, penetiance >95%.
Loss-of-function mutations in the ÀTP2À2 gene
encoding saico/endoplasmic ieticulum calcium
adenosine tiiphosphatase isofoim 2 (SERCA 2),
which impaii intiacellulai Ca2- signaling.
Frecìpìtatìo¿ Factors Fiequently woise in sum-
mei with heat and humidity as majoi factois; can
be exaceibated by UVB, mechanical tiauma, bac-
teiial infections. Often associated with affective
disoideis and iaiely with decieased intelligence.
CIINICAI MANIF£SIAII0N
Usually insidious; onset is abiupt aftei piecipi-
tating factois; associated with seveie piuiitus
and often pain.
Skìo Iesìoos Multiple disciete scaling of ciusted,
piuiitic papules (Fig. 5-2); when scaling ciust is
iemoved, a slitlike opening becomes visible (Fig.
5-3). Confluence to laige plaques coveied by
hypeitiophic waity masses that aie foul smelling,
paiticulaily in inteitiiginous aieas.
DIstrIhutIvn Coiiesponding to the °seboi-
iheic aieas": chest (Fig. 5-2), back, eais, na-
solabial folds, foiehead (Fig. 5-3), scalp; axilla,
neck, gioin.
FICük£ 5-2 0arìer dìsease: chest |||r+|, |eº|ouº +|e |edd|º|·||oWu, ºc+||uç +ud c|uº|ed p+pu|eº ||+| |ee|
W+||, W|eu º||o|ed. w|e|e c|uº|º |+.e |eeu |ero.ed ||e|e +|e º||||||e e|oº|ouº ||+| +|e |+|e| co.e|ed |, |ero|·
||+ç|c c|uº|º.
S£CII0N 5 \|'CE||A|E0u' E|||Ek\A| ||'0k|Ek' 91
Fa|ms aod So|es Multiple, flat, cobblestone-
like papules.
Appeoda¿es Haii not involved, but peimanent
alopecia may iesult fiom extensive scalp involve-
ment. Nails thin, splitting distally and showing
chaiacteiistic V-shaped scalloping (see Fig. 33-31).
Mucous Membraoes White, centially de-
piessed papules on mucosa of cheeks, haid and
soft palate, and gums, °cobblestone" lesions.
0IS£AS£ ASS0CIAII0N
Associated with atro|eraìosís ·errutí[ormís,
allelic with DD. Multiple, small flat-topped
papules piedominantly on doisa of hands
and feet.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Dyskeiatotic cells in the
spinous layei (coips ionds) and stiatum coi-
neum (giains), supiabasal acantholysis and
clefts (lacunae), papillaiy oveigiowth of the
epideimis and hypeikeiatosis.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Diagnosis based on histoiy of familial involve-
ment, clinical appeaiance, and histopathology.
May be confused with seboiiheic deimatitis,
Giovei disease, benign familial pemphigus
(Hailey-Hailey disease), and pemphigus fo-
liaceus. Aciokeiatosis veiiucifoimis: flat waits
(veiiucae planae juveniles).
C0ükS£ AN0 Fk0CN0SIS
Peisisting thioughout life, not associated with
cutaneous malignancies.
MANAC£M£NI
Sunscieens to avoid UV-induced exacei-
bations, avoidance of fiiction and iubbing
(tuitle neck sweateis), antibiotic theiapy
(systemic and topical) to suppiess bacteiial
infection, topical ietinoids (tazaiotene and
adapalene) oi systemic ietinoids (isotietinoin
oi acitietin). Systemic theiapy can be modi-
fied accoiding to seasonal vaiiation of the
disease.
FICük£ 5-3 0arìer dìsease: Iorehead |+|||, co+|eºc|uç, |,pe||e|+|o||c p+pu|eº ||+| +|e e|oded +ud
c|uº|ed. I|e r+|u couce|u o| |||º ,ouuç |er+|e W+º d|º||çu|ereu|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 92
£FI0£MI0I0C¥
A¿e oI 0oset Middle age and oldei, mean age
50 yeais.
Sex Males > females.
Frecìpìtatìo¿ Factors Heavy, sweat-inducing ex-
eicise, excessive solai exposuie, exposuie to heat,
and peisistent fevei; may also occui in bediid-
den patients, with heat and sweating as factois.
CIINICAI MANIF£SIAII0N
Usually abiupt onset of piuiitus and simul-
taneous appeaiance of ciops of lesions.
Skìo Iesìoos Skin-coloied, pink oi ieddish
papules (small, 3 to 5 mm, some with slight
scale oi smooth) (Fig. 5-4), papulovesicles, and
eiosions. Veiy similai to Daiiei disease. Upon
palpation, smooth oi waity. Scatteied, disciete
on cential tiunk (Fig. 5-4) and pioximal
extiemities.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Acantholysis and spongi-
osis, focal acantholytic dyskeiatosis occuiiing at
the same time and simulating Daiiei disease,
pemphigus foliaceus, and Hailey-Hailey disease;
in the deimis theie is a supeificial infiltiate of
eosinophils, lymphocytes, and histiocytes.
A p|u||||c de|r+|oº|º |oc+|ed p||uc|p+||, ou ||e
||uu|, occu|||uç +º c|opº o| d|ºc|e|e p+pu|+| o|
p+pu|o.eº|cu|+| |eº|ouº, ºp+|ºe |o uure|ouº.
0ccu|º |u +du||º.
||u|||uº |º r+|u º,rp|or.
uºu+||, ||+uº|eu| |u| + pe|º|º|eu| |o|r |º
|ecoçu|/ed.
|||uc|p+| ||º|op+||o|oç|c |e+|u|e. .+||+||e |oc+|
+c+u||o|,º|º +ud d,º|e|+|oº|º.
|o e.|deuce o| çeue||c p|ed|ºpoº|||ou.
',uou,r. ||+uº|eu| +c+u||o|,||c de|r+|oº|º.
Ck0v£k 0IS£AS£ (C0) |C|·9 . 102.3

|C|·¹0 . | ¹¹.¹
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Often difficult.
Sma|| 0ìscrete Frurìtìc Fapu|es oo Chest Daiiei
disease, heat iash (miliaiia iubia), papulai
uiticaiia, scabies, deimatitis heipetifoimis
(heie theie is giouping and the lesions aie sym-
metiic), Pityiospoium oi eosinophilic folliculi-
tis, insect bites, and diug eiuptions.
C0ükS£ AN0 Fk0CN0SIS
The disease is by no means always tiansient, and
theie appeai to be two types: acute (°tiansient")
and chionic ielapsing. The mean duiation in
one seiies was 47 weeks.
MANAC£M£NI
Iopìca| Class I topical glucocoiticoids undei
plastic (e.g., diy-cleaning plastic suit bags with
holes cut foi aims) aie used foi 4 h.
Systemìc Oial glucocoiticoids and dapsone
have been used with success, but ielapses occui
aftei withdiawal.
Fhototherapy UVB oi PUVA photochemo-
theiapy may be useful foi patients who do not
iespond to topical glucocoiticoids undei occlu-
sion. Isotietinoin has been used in iefiactoiy
cases.
S£CII0N 5 \|'CE||A|E0u' E|||Ek\A| ||'0k|Ek' 93
CIASSIFICAII0N
1
Iype 1: C|assìc Adu|t Geneialized, beginning
on head and neck.
Iype 2: Atypìca| Adu|t Geneialized, spaise
haii.
Iype 3: C|assìc luveoì|e Appeais within the
fiist two yeais of life, geneialized.
Iype 4: Cìrcumscrìbed luveoì|e In piepubeital
childien, localized.
Iype 5: Atypìca| luveoì|e Onset in fiist few
yeais of life, familial, geneialized.
1
W.A.D. Giiffiths Pityiiasis iubia pilaiis. Clin Exp
Deimatol 5:105, 1980; and A. Gonzales-López et al:
Bi J Deimatol 140:931, 1999.
1
W.A.D. Giiffiths Pityiiasis iubia pilaiis. Clin Exp
Deimatol 5:105, 1980; and A. Gonzales-López et al:
Bi J Deimatol 140:931, 1999.
k+|e, c||ou|c, p+pu|oºqu+rouº d|ºo|de| o||eu
p|oç|eºº|uç |o e|,|||ode|r+.
'|\ |,peº e\|º|.
|o|||cu|+| |,pe||e|+|o||c p+pu|eº, |edd|º|·o|+uçe
p|oç|eºº|uç |o çeue|+||/ed e|,|||ode|r+. '|+|p|,
der+|c+|ed |º|+udº o| uu+||ec|ed (uo|r+|)
º||u.
w+\,, d|||uºe, o|+uçe |e|+|ode|r+ o| p+|rº +ud
ºo|eº, u+||º r+, |e +||ec|ed.
\oº| e||ec||.e ||e|+p, |º re||o||e\+|e, |e||uo|dº.
FII¥kIASIS kü8kA FIIAkIS (FkF)
|C|·9 . o9o.+

|C|·¹0 . | ++.+   
Iype 6: hIv-Assocìated Geneialized, associ-
ated with acne conglobata, hidiadenitis sup-
puiativa, and lichen spinulosus.
£FI0£MI0I0C¥
Estimated incidence 1: 5000 and in 1 in 15,000
deimatology patients. Bimodal distiibution with
a peak incidence in the fiist and fifth decades of
life. Affects both sexes and occuis in all iaces.
£II0I0C¥ AN0 FAIh0C£N£SIS
Unknown. A dysfunction in vitamin A metabo-
lism has been suggested but not pioven. Genetic
FICük£ 5-4 Crover dìsease A |+º| couº|º||uç o| |edd|º|, |,pe||e|+|o||c ºc+||uç +ud/o| c|uº|ed p+pu|eº
W||| + º+udp+pe| |ee| upou p+|p+||ou. |+pu|eº +|e d|ºc|e|e, ºc+||e|ed ou ||e ceu||+| ||uu| +ud .e|, p|u||||c.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 94
factois aie believed to play a ciitical iole in the
induction of PRP.
CIINICAI MANIF£SIAII0N
Both insidious and iapid onset occui.
Skìo Iesìoos All types of PRP. An eiuption
of folliculai hypeikeiatotic papules of ieddish-
oiange coloi usually spieading in a cephalocaudal
diiection (Fig. 5-5). Confluence to a ieddish-
oiange psoiiasifoim, scaling deimatitis with
shaiply demaicated islands of unaffected skin
(Fig. 5-6). Piogiession to eiythiodeima (except
foi type 2 and type 4) (Fig. 5-7).
DIstrIhutIvn Types 1, 2, 3, 5, and 6: Geneial-
ized, classically beginning on the head and neck,
then spieading caudally.
Sca|p aod haìr Scalp affected, as in psoiiasis,
often leading to asbestos-like accumulation of
scale. Haii not affected except in type 2 wheie
spaise scalp haii is obseived.
Mucous Membraoes Spaied.
Naì|s Common but not diagnostic. Distal
yellow-biown discoloiation, nail plate thick-
ening, subungual hypeikeiatosis, and splintei
hemoiihages. See section 33.
Assocìated Coodìtìoos Ichthyosifoim lesions
on legs in type 2. Scleiodeima-like appeaiance
of hands and feet in type 5. Acne conglobata,
hidiadenitis suppuiativa, and lichen spinulosus
in type 6.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
The diagnosis is made on clinical giounds. The
diffeiential diagnosis includes psoiiasis, follicu-
lai ichthyosis, eiythiokeiatodeima vaiiabilis,
nonbullous ichthyosifoim eiythiodeima.
IA80kAI0k¥ £XAMINAII0NS
Chemìstry No diagnostic featuies.
hìstopatho|o¿y Not diagnostic. Suggestive:
Hypeikeiatosis, acanthosis with bioad shoit iete
iidges, alteinating autokeiatosis and paiakeia-
tosis. Keiatinous plugs of folliculai infundibula
and peiifolliculai aieas of paiakeiatosis may be
piesent. Piominent gianulai layei may distin-
guish PRP fiom psoiiasis. Supeificial peiivas-
culai lymphocytic infiltiate.
C0ükS£ AN0 Fk0CN0SIS
A socially and psychologically disabling con-
dition. Long duiation; type 3 often iesolves
aftei 2 yeais; type 4 may cleai. Type 5 has a veiy
chionic couise. Type 6 may iespond to highly
active antiietioviial theiapy (HAART).
MANAC£M£NI
Topical theiapies consist of emollients, keia-
tolytic agents, vitamin D
3
(calcipioptiiol),
FICük£ 5-5 Fìtyrìasìs rubra pì|arìs (type 1, c|assìc adu|t) |u|||+| |eº|ouº +|e d|ºc|e|e, d|ººer|u+|ed |o|·
||cu|+| |,pe||e|+|o||c p+pu|eº o| |edd|º|·o|+uçe co|o|.
S£CII0N 5 \|'CE||A|E0u' E|||Ek\A| ||'0k|Ek' 95
glucocoiticoids, and vitamin A analogues
(tazaioten). All not veiy effective. Photothei-
apy ¦ultiaviolet A phototheiapy, naiiowband
ultiaviolet B phototheiapy, and photochemo-
theiapy (PUVA)] aie effective in some cases.
Most effective tieatment consists of systemic
administiation of methotiexate oi ietinoids
(both as in psoiiasis). In type 6 HAART. The
anti-TNF agents, e.g., iemicade and enbiel, aie
effective.
FICük£ 5-6 0arìer dìsease (type 1, c|assìc adu|t) Ceue|+||/ed p+pu|eº |eç|uu|uç ou ||e |e+d +ud
uec| |+.e co+|eºced ou ||e c|eº| o| + 51·,e+|·o|d r+|e W|o |+d |+|eu eº||oçeuº. I|e|e +|e º|+|p|, der+|c+|ed
|º|+udº o| uu+||ec|ed uo|r+| º||u.
FICük£ 5-T Fìtyrìasìs rubra pì|arìs (type 1, c|assìc adu|t) 0|+uçe·|edd|º| p+pu|eº |+.e co+|eºced |o
ue+| e|,|||ode|r+, ºp+||uç |ºo|+|ed |º|+udº o| uo|r+| º||u. A|ºo uo|e e|,|||ode|r+, |u.o|.ereu| o| ||e |+udº |u
|||º 55·,e+|·o|d Wor+u.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 96
|'A| |º ||e roº| corrou |o|r o| ||e po|o|e|+·
|oºeº (|o| c|+ºº|||c+||ou ºee po|o|e|+|oº|º p|c|u|e
ç+||e|, |u ||e ou||ue .e|º|ou.)
uu||o|r|, ºr+||, +uuu|+| ||+| p+pu|eº |+uç|uç
||or 2 |o 5 rr |u d|+re|e|.
||º||||u|ed º,rre|||c+||, ou ||e e\||er|||eº +ud
|oc+|ed p|edor|u+u||, |u ºuu·e\poºed º||eº.
I,p|c+||, ºp+|e p+|rº, ºo|eº, +ud rucouº rer·
||+ueº.
C|+|+c|e||º||c |e+|u|e. We||·der+|c+|ed |,pe||·
e|+|o||c |o|de| o| |ud|.|du+| |eº|ouº, uºu+||, · ¹
rr |u |e|ç|| W||| + c|+|+c|e||º||c |ouç||ud|u+|
|u||oW euc||c||uç ||e eu|||e |eº|ou (||çº. 5·3
+ud 5·9).
Aº |eº|ouº p|oç|eºº, ||e ceu||+| +|e+ |ecoreº
+||op||c +ud uu||d|o||c.
',rp|orº. +º,rp|or+||c o| r||d|, p|u||||c coº·
re||c+||, d|º||çu||uç .
Ieudº |o |e |u|e|||ed +º +u +u|oºor+| dor|u+u|
d|ºo|de|.
|+||oçeueº|º uu|uoWu.
A |eu|çu coud|||ou, |u| |+|e|, + p|ecu|ºo| |o| |u
º||u o| |u.+º|.e ºqu+rouº ce|| c+|c|uor+.
I|e+|reu|. |op|c+| 5·||uo|ou|+c||, |e||uo|dº, +ud |r·
|qu|rod. Iop|c+| |e||uo|dº r+, |rp|o.e |eº|ouº.
|+||eu|º º|ou|d |e rou||o|ed |o| 'CC.
|C|·9 . o92.15

|C|·¹0 . 0 32.3
0ISS£MINAI£0 SüF£kFICIAI ACIINIC F0k0k£kAI0SIS (0SAF)
FICük£ 5-8 0ìssemìoated superIìcìa| actìoìc porokeratosìs \e|, ||+| +uuu|+| p+pu|eº W||| + .e|,
c|+|+c|e||º||c |,pe||e|+|o||c |o|de| ºu||ouud|uç ||e |eº|ou. |ud|.|du+| |eº|ouº |+.e co+|eºced |o |||eçu|+| p+|c|eº,
W||c| +|e +|ºo ºu||ouuded |, ||e po|o|e|+|o||c |o|de|. I||º |º ou ||e |oWe| +|r o| + 55·,e+|·o|d |er+|e W|o |+d
|eeu c||ou|c+||, ºuu·e\poºed |o| dec+deº. I|e e|up||ou W+º d|º||||u|ed º,rre|||c+||, ou +|rº +ud |eçº |u| ou|,
|u ºuu·e\poºed º||eº.
S£CII0N 5 \|'CE||A|E0u' E|||Ek\A| ||'0k|Ek' 9T
FICük£ 5-9 0ìssemìoated superIìcìa| actìoìc porokeratosìs 'r+|| +uuu|+| ||+| p+pu|eº up |o + rr |u
d|+re|e| ºu||ouuded |, + We||·der+|c+|ed |,pe||e|+|o||c |o|de|. w||| + |+ud |euº ||e |ouç||ud|u+| |u||oW euc||·
c||uç ||e eu|||e |eº|ou c+u |e ºeeu.
98
8üII0üS 0IS£AS£S
S E C I | 0 N 6
CIASSIFICAII0N
Based on level of cleavage and blistei foimation
theie aie thiee main types:
· Epideimolytic. Cleavage occuis in keiatino-
cytes: EB simplex (EBS)
· Junctional. Cleavage occuis in basal lamina:
junctional EB (JEB)
· Deimatolytic. Cleavage occuis in most su-
peificial papillaiy deimis: deimolytic, oi dys-
tiophic, EB (DEB)
In each of these gioups theie aie seveial distinct
types of EB based on clinical, genetic, his-
tologic/electionmicioscopic, and biochemical
evaluation (Table 6-1). Only the most impoi-
tant aie discussed heie.
£FI0£MI0I0C¥
The oveiall incidence of heieditaiy EB is placed
at 19.6 live biiths pei 1 million biiths in the
United States. Stiatified by subtype, the inci-
dences aie 11 foi EBS, 2 foi JEB, and 5 foi DEB.
The estimated pievalence in the United States is
8.2 pei million, but this figuie iepiesents only
the most seveie cases as it does not include
the majoiity of veiy mild disease going unie-
poited.
A ºpec||ur o| |+|e çeuode|r+|oºeº |u W||c| +
d|º|u||ed co|e|euce o| ||e ep|de|r|º +ud/o| de|·
r|º |e+dº |o |||º|e| |o|r+||ou |o||oW|uç ||+ur+.
neuce, ||e deº|çu+||ou ¤-:|c¤¤·|±||¤±º !-·¤c·
|¤º-º
||ºe+ºe r+u||eº|+||ouº |+uçe ||or .e|, r||d |o
ºe.e|e|, ru|||+||uç +ud e.eu |e||+| |o|rº ||+| d||·
|e| |u rode o| |u|e|||+uce, c||u|c+| r+u||eº|+||ouº,
+ud +ººoc|+|ed ||ud|uçº.
C|+ºº|||c+||ou |+ºed ou ||e º||e o| |||º|e| |o|r+||ou
d|º||uçu|º|eº |||ee r+|u ç|oupº. ep|de|ro|,||c
o| EB º|rp|e\ (EB'), juuc||ou+| EB (lEB), +ud
de|ro|,||c, o| d,º||op||c, EB (|EB).
|u e+c| o| ||eºe ç|oupº ||e|e +|e ºe.e|+| d|º||uc|
|,peº o| EB |+ºed ou c||u|c+|, çeue||c, ||º|o|oç|c,
+ud ||oc|er|c+| e.+|u+||ou.
|C|·9 . 151.!9
°
|C|·¹0 . 0 3¹
h£k£0IIAk¥ £FI0£kM0I¥SIS 8üII0SA (£8)
£II0I0C¥ AN0 FAIh0C£N£SIS
Ceoetìc 0eIects Molecules involved aie listed
in Table 6-1 and localization in the tissue and
sites of cleavage aie shown in Image 6-1.
CIINICAI Fh£N0I¥F£S
£8 Sìmp|ex (£8S)
A tiauma-induced, intiaepideimal blisteiing,
based in most cases on mutations of the genes
foi keiatins 5 and 14 iesulting in a distuibance
of the stability of the keiatin filament netwoik
(Table 6-1). This causes cytolysis of basal keiati-
nocytes and a cleft in the basal cell layei (Image
6-1). Diffeient subgioups have consideiable
phenotypic vaiiations (Table 6-1), and theie aie
eight distinct foims, most of which aie domi-
nantly inheiited. The two most common aie
desciibed below.
Generu|Ized EBS (Table 6-1) The so-called
Koebnei vaiiant is dominantly inheiited, with
onset at biith to eaily infancy. Theie is geneial-
ized blisteiing following tiauma with a piedi-
lection foi tiaumatized body sites such as feet,
hands, elbows, and knees. Blisteis aie tense oi
flaccid at fiist and lead to eiosions (Fig. 6-1).
Theie is iapid healing and only minimal scai-
iing at sites of iepeated blisteiing. Palmoplantai
S£CII0N 6 Bu||0u' ||'EA'E' 99
FICük£ 6-1 Ceoera|ìted £8S I||º +·,e+|· o|d ç||| |+º |+d |||º|e||uç º|uce .e|, e+||, |u|+uc, W||| p|ed||ec·
||ou |o| ||+ur+||/ed |od, º||eº ºuc| +º p+|rº +ud ºo|eº |u| +|ºo e||oWº +ud |ueeº. B||º|e||uç +|ºo occu|º |u o||e|
+|e+º ºuc| +º ||e |o|e+|r, +º º|oWu |e|e, +ud ou ||e ||uu|. |o|e ||+| deºp||e ru|||p|e |||º|e||uç ep|ºodeº ||e|e |º
|+|d|, +u, e.|deuce o| ºc+|||uç ou ||e p+|rº o| |||º c|||d.
IMAC£ 6-1 |oc+||/+||ou o| |+|çe| +d|eº|ou º||eº +ud c|e|| |o|r+||ou |u ºe|ec|ed |e|ed||+|, +ud +u|o|rruue
|u||ouº d|ºe+ºeº. (\od|||ed ||or ||ç. !0·5 |u l| Bo|oçu|+ e| +|. |-·¤c|¤|¤¸,, \oº|,, |oudou, ||||+de|p||+, 200!,
W||| pe|r|ºº|ou.)
EBA
EBS
PV
Desmosome
DGL 1,3
BPAG2
collagen
Type
VII
Laminin 5
BPAG1
Basal
Keratinocyte
Hemidesmosome
Lamina lucida
Sublamina
densa region
Lamina densa
DEB
BP, PG, LAD, CP
GABEB, HERLITZ
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 100
hypeikeiatoses may be piesent. Nails, teeth, and
oial mucosa aie usually spaied.
Lvcu|Ized EBS Webei-Cockayne subtype
(Table 6-1). This is the most common foim of
EBS. Onset in childhood oi latei. The disease
may not piesent itself until adulthood, when
thick-walled blisteis on the feet and hands oc-
cui aftei excessive exeicise, manual woik, oi
militaiy tiaining (Fig. 6-2). Incieased ambient
tempeiatuie facilitates lesions. Hypeihidiosis
of palms and soles is associated, and secondaiy
infection of blisteied lesions often occuis.
luoctìooa| £8 (l£8)
All foims of JEB shaie the pathologic featuie of
blistei foimation within the lamina lucida of the
basement membiane (Image 6-1). Mutations
aie in the gene foi collagen XVII and laminin
(Table 6-1). This tiait is autosomal iecessive
and compiises clinical phenotypes depending
on the type of genetic lesion and enviionmental
IA8I£ 6-1 C|assification of Epidermo|ysis Bu||osa
Ieve| oI Separatìoo 0ìsease 0eIect
'|rp|e\ Ceue|+||/ed/Koe|ue| KkI5/KkI¹+
'|rp|e\ ne|pe|||o|r|º/|oW||uç·\e+|+ KkI5/KkI¹+
'|rp|e\ |oc+||/ed/we|e|·Coc|+,ue KkI5/KkI¹+
'|rp|e\ 0çu+ KkI5/KkI¹+
'|rp|e\ 'upe|||c|+||º KkI5/KkI¹+
'|rp|e\ \o|||ed p|çreu|+||ou KkI5
ner|deºroºor+|
c
EB W||| ruºcu|+| d,º||op|, ||EC¹
ner|deºroºor+|
|
EB W||| p,|o||c +||eº|+ |ICB+/|ICAo
luuc||ou+| C|+.|º/ne||||/ |A\B!/|A\A!/|A\C2
luuc||ou+| \|||º |A\B!/|A\A!/|A\C2
luuc||ou+|
c
Ceue|+||/ed +||op||c |eu|çu C0|¹1A¹/|A\B!
luuc||ou+| |oc+||/ed C0|¹1A¹
|,º||op||c |+º|u| C0|1A¹
|,º||op||c Coc|+,ue·Iou|+|ue C0|1A¹
|,º||op||c |oc+||/ed k|EB C0|1A¹
|,º||op||c n+||ope+u·'|ereuº C0|1A¹
\+||+||e K|ud|e| º,ud|ore K|||¹
|eºroºor+| Ec|ode|r+| d,ºp|+º|+·º||u ||+ç||||, |K|¹
C0|1A¹, co||+çeu, |,pe \||,
¹
, EB, ep|de|ro|,º|º |u||oº+, |ICB, |u|eç||u , KkI, |e|+||u, |A\A, |+r|u|u , |A\B, |+r|u|u , |K|, p|+|op||||u,
||EC, p|ec||u, k|EB, |eceºº|.e d,º||op||c ep|de|ro|,º|º |u||oº+.
c
A||e|u+||.e|, c|+ºº|||ed +º º|rp|e\.
|
A||e|u+||.e|, c|+ºº|||ed +º juuc||ou+|.
:
C+ºe W||| |,pe /\|| co||+çeu c,|op|+ºr|c de|e||ou º|oWed |o|| juuc||ou+| +ud |er|deºroºor+| |e.e|º o| º||u ºep+|+||ou.
'ou|ce. ||or \.|. \+||u|o.|c|, EA B+ue|. |u|e|||ed ep|de|ro|,º|º |u||oº+, |u K wo||| e| +|. (edº.). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-
1
||
ed|||ou. |eW \o||, \cC|+W·n|||, 2003
factois. The thiee piincipal subtypes (see Table
6-1) aie desciibed below.
JEB GruvIs (Her|Itz EB) Patients often do
not suivive infancy; the moitality iate is 40%
duiing the fiist yeai of life. Theie is geneial-
ized blisteiing at biith (Fig. 6-3) oi clinically
distinctive and seveie peiioiificial gianulation,
loss of nails, and involvement of most mu-
cosal suifaces. The skin of these childien may
be completely denuded, iepiesenting oozing
painful eiosion. Associated findings include all
symptoms iesulting fiom geneialized epithelial
blisteiing with iespiiatoiy, gastiointestinal, and
genitouiinaiy oigan systems involved.
JEB MItIs These childien may have modeiate
oi seveie JEB at biith but suivive infancy and
clinically impiove with age. Peiioiificial non-
healing eiosions duiing childhood.
Generu|Ized AtrvphIc BenIgn EpIdermv|ysIs
Bu||vsu (GABEB) GABEB is a sepaiate JEB
that piesents at biith with geneialized cutaneous
S£CII0N 6 Bu||0u' ||'EA'E' 101
FICük£ 6-2 Ioca|ìted £8S I||c|·W+||ed |||º|e|º ou ||e ºo|eº. I|e d|ºe+ºe p|eºeu|ed ||ºe|| |o| ||e |||º| ||re
du||uç r||||+|, ||+|u|uç W|eu |||º ¹9·,e+|·o|d r+u |+d |o r+|c| o.e| + |ouç d|º|+uce.
FICük£ 6-3 luoctìooa| epìdermo|ysìs bu||osa (her|ìtt varìaot) I|e|e +|e |+|çe e|oded, oo/|uç +ud
||eed|uç +|e+º ||+| occu||ed |u||+p+||ur. w|eu |||º ueW|o|u |º ||||ed up, d|º|odçreu| o| ep|de|r|º +º We|| +º
e|oº|ouº occu| W||| r+uu+| |+ud||uç.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 102
blisteiing (Fig. 6-4) and eiosions not only on
the extiemities but also on the tiunk, face, and
scalp. Suivival to adulthood is the iule, but blis-
teiing on tiaumatized aieas continues (Fig. 6-
5). It is paiticulaily pionounced with incieased
ambient tempeiatuie, and theie is atiophic
healing of the lesions. Nail dystiophy, nonscai-
iing oi scaiiing alopecia, mild oial mucous
membiane involvement; enamel defects may
occui. Mutations aie in the genes foi collagen
type XVII and laminin (Table 6-1).
0ystrophìc £pìdermo|ysìs 8u||osa ( 0£8 )
A spectium of deimolytic diseases wheie blis-
teiing occuis below the basal lamina (Image
6-1); healing is theiefoie usually accompanied
by scaiiing and milia foimation-hence, the
name Jysìro¡|ít . Theie aie foui piincipal sub-
types, and all aie due to mutations in anchoiing
fibiil type VII collagen (Table 6-1). Anchoiing
fibiils aie theiefoie only iudimentaiy oi absent.
Of the foui main types of DEB, only two aie
desciibed below.
DvmInunt DEB Cockayne-Touiaine disease.
Onset in infancy oi eaily childhood with acial
blisteiing and nail dystiophy; milia and scai
foimation, which may be hypeitiophic oi hy-
peiplastic. Oial lesions aie uncommon, and
teeth aie usually noimal.
RecessIve DEB (RDEB) Compiises a laigei
spectium of clinical phenotypes. The localized,
less seveie foim (RDEB mitis) occuis at biith,
shows acial blisteiing, atiophic scaiiing, and
little oi no mucosal involvement. Geneialized,
seveie RDEB, the Hallopeau-Siemens vaiiant,
is mutilating. Theie is geneialized blisteiing at
biith, and piogiession and iepeated blisteiing
at the same sites (Fig. 6-6) iesult in iemaikable
scaiiing and ulceiations, syndactyly with loss
of nails (Fig. 6-7) and even mitten-like defoim-
ities of hands and feet, flexion contiactuies.
Theie aie enamel defects with caiies and paio-
dontitis, stiictuies and scaiiing in the oial mu-
cous membiane and esophagus, uiethial and
anal stenosis, and oculai suiface scaiiing; also
malnutiition, giowth ietaidation, and anemia.
The most seiious complication is squamous cell
caicinoma in chionic iecuiient eiosions.
0IACN0SIS
Based on clinical appeaiance and histoiy. His-
topathology deteimines the level of cleavage,
which is fuithei defined by election micios-
copy and/oi immunohistochemical mapping.
Westein blot, Noithein blot, iestiiction fiag-
ment length polymoiphism (RFLP) analysis,
and DNA sequences may then identify the
mutated gene.
MANAC£M£NI
Theie is as yet no causal theiapy foi EB, but
gene theiapy is being investigated. Management
is tailoied to the seveiity and extent of skin in-
volvement and consists of suppoitive skin caie,
suppoitive caie foi othei oigan systems, and
systemic theiapies foi complications. Wound
management, nutiitional suppoit, and infec-
tion contiol aie key to the management of all
EB patients.
In EBS, maintenance of a cool enviionment
and use of soft, well-ventilated shoes, aie im-
poitant. Blisteied skin is tieated by saline com-
piesses and topical antibiotics oi, in the case of
inflammation, with topical steioids. Moie se-
veiely affected JEB and DEB patients aie tieated
like patients in a buin unit. Gentle bathing and
cleansing aie followed by piotective emollients
and nonadheient diessings.
Management of cutaneous infection is im-
poitant, and suigical tieatment is often ie-
quiied in DEB foi the ielease of fused digits and
coiiection of limb contiactuies.
Although iaie, EB and, in paiticulai, JEB and
DEB pose a majoi health and socioeconomic
pioblem. Oiganizations such as the Dystiophic
Epideimolysis Bullosa Reseaich Association
(DEBRA) offei assistance that includes patient
education and suppoit.
S£CII0N 6 Bu||0u' ||'EA'E' 103
FICük£ 6-5 Ceoera|ìted atrophìc beoì¿o epìdermo|ysìs bu||osa (CA8£8) I||º 20·,e+|·o|d r+u |+º
|+d çeue|+||/ed cu|+ueouº |||º|e||uç º|uce |||||. |o|e. e|oº|ouº ou ||e |e|| |oWe| |+c| +ud |ero|||+ç|c c|uº|º ou
||e |oWe| +|rº. E|,||er+ ou ||e |+c| |ud|c+|eº º||eº o| p|e.|ouº |||º|e||uç.
FICük£ 6-4 Ceoera|ìted atrophìc beoì¿o epìdermo|ysìs bu||osa (CA8£8) I||º ¹9·,e+|·o|d r+u |+º |+d
cu|+ueouº |||º|e||uç º|uce |||||, W||| |||º|e|º +ud e|oº|ouº +||º|uç ou ||e e||oWº +ud |ueeº |u| +|ºo ou ||e ||uu| +ud +|rº
|o||oW|uç ||+ur+. |o|e. |||·de||ued e|,||er+º +| º||eº o| p|e.|ouº |||º|e||uç. I|e|e |º uo ºc+|||uç |u| ºore ºpo||, +||op|,.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 104
FICük£ 6-6 Ceoera|ìted recessìve dystrophìc epìdermo|ysìs bu||osa (k0£8) |u |||º ºe.e|e d|ºe+ºe
|||º|e||uç occu|º o||eu +| ||e º+re º||eº, +º |u |||º ¹0·,e+|·o|d ç|||. B||º|e|º |e+d |o e|oº|ouº +ud ||eºe |ecore
u|ce|º ||+| |+.e + |oW |eudeuc, |o |e+|. w|eu |e+||uç occu|º || |eºu||º |u ºc+|||uç. I||º ç||| +|ºo |+º eu+re|
de|ec|º W||| c+||eº, º|||c|u|eº o| ||e eºop|+çuº, ºe.e|e +uer|+, +ud couº|de|+||e ç|oW|| |e|+|d+||ou. || |º o|.|ouº
||+| ||eºe |+|çe Wouudº +|e po||+| eu|||eº |o| º,º|er|c |u|ec||ou.
FICük£ 6-T Ceoera|ìted recessìve dystrophìc epìdermo|ysìs bu||osa (k0£8) |oºº o| +|| ||uçe|u+||º,
º,ud+c|,|,, +ud ºe.e|e +||op||c ºc+|||uç ou ||e do|º+ o| ||e |+ud.
S£CII0N 6 Bu||0u' ||'EA'E' 105
|+r|||+| |eu|çu perp||çuº, o| n+||e,·n+||e, d|º·
e+ºe, |º + |+|e çeuode|r+|oº|º W||| dor|u+u| |u·
|e|||+uce ||+| |º c|+ºº|c+||, deºc|||ed +º + |||º|e||uç
d|ºo|de| |u| +c|u+||, p|eºeu|º +º +u e|,||er+|ouº,
e|oº|.e, oo/|uç coud|||ou W||| c|+c|º +ud ||ººu|eº
|oc+||/ed |o ||e u+pe o| ||e uec|, +\|||+e (||ç. o·3).
'u|r+rr+|, |eç|ouº, |uçu|u+| |o|dº, +ud ºc|o·
|ur c·- ¤c·¤· º·|-º ¤| ·¤.¤|.-¤-¤|.
I|e uude||,|uç p+||o|oç|c p|oceºº |º +c+u||o|,º|º
W|e|e|, ||e ||+ç||||, o| ||e ep|de|r|º |º due |o +
de|ec| |u ||e +d|eº|ou corp|e\ |e|Weeu deºro·
ºor+| p|o|e|uº +ud |ouo|||+reu|º.
I|e çeue||c +|uo|r+|||, ||eº |u 1í|2C|, W||c|
eucodeº +u AI|·poWe|ed c+|c|ur purp.
0uºe| |º uºu+||, |e|Weeu ||e ||||d +ud |ou|||
dec+deº.
0||eu r|º|+|eu |o| |u|e||||ço, c+ud|d|+º|º, o| |||c·
||ou+| o| cou|+c| de|r+||||º.
|ud|.|du+| |eº|ouº couº|º| o| r|c|oºcop|c+||, ºr+||
||+cc|d .eº|c|eº ou +u e|,||er+|ouº |+c|ç|ouud
||+| ºoou |u|u |u|o e|oded p|+queº W||| ||e de·
ºc|||ed, ||ç||, c|+|+c|e||º||c, ||ººu|ed +ppe+|+uce
(||ç. o·3).
C|uº||uç, ºc+||uç, +ud |,pe|||op||c .eçe|+||.e
ç|oW||º r+, occu|.
n|º|o|oç, e\p|+|uº ||e c||u|c+| +ppe+|+uce +º
ep|de|r+| ce||º |oºe ||e|| co|e|euce W||| +c+u·
||o|,º|º |||ouç|ou| ||e ep|||e||ur, ç|.|uç ||e
+ppe+|+uce o| + d||+p|d+|ed |||c| W+||.
Co|ou|/+||ou o| ||e |eº|ouº, p+|||cu|+||, |, '|c¤|,·
|¤:¤::±º c±·-±º |º + |||ççe| |o| |u|||e| +c+u||o|,·
º|º +ud r+|u|eu+uce o| ||e p+||o|oç|c p|oceºº.
'ecoud+|, co|ou|/+||ou |, Cc¤!·!c |+º + º|r||+|
e||ec|.
I|e+|reu| |eº|º ou +u||r|c|o||+| ||e|+p,,
+dr|u|º|e|ed |o|| |op|c+||, +ud º,º|er|c+||,,
º,º|er|c+||,, ||e |e||+c,c||ueº ºeer |o Wo||
|e||e| ||+u roº|. \up||oc|u |op|c+||,. Iop|c+|
ç|ucoco|||co|dº dep|eºº ||e +u||·|u||+rr+|o|,
|eºpouºe +ud +cce|e|+|e |e+||uç. |u ºe.e|e
c+ºeº, de|r+||+º|ou o| c+||ou d|o\|de |+ºe|
.+po||/+||ou |e+dº |o |e+||uç W||| ºc+|º, W||c| +|e
|eº|º|+u| |o |ecu||euceº. I|e coud|||ou |ecoreº
|eºº ||ou||eºore W||| +çe.
|C|·9 . o9+.5
°
|C|·¹0 . 0 32.3
FAMIIIAI 8£NICN F£MFhICüS
FICük£ 6-8 Famì|ìa| beoì¿o pemphì¿us I||º +o·,e+|·o|d r+|e |+º |+d oo/|uç |eº|ouº |u |o|| +\|||+e,
occ+º|ou+||, |u ||e ç|o|uº +ud ºore||reº +|ºo ou ||e u+pe o| ||e uec|, |o| ºe.e|+| ,e+|º. E|up||ouº Wo|ºeu du||uç
||e ºurre| rou||º. I|e |+||e| +ud º|º|e| |+.e º|r||+| |eº|ouº ||+| W+\ +ud W+ue. |eº|ouº +|e p+|u|u| +ud º|oW
|,p|c+| c|+c|º +ud ||ººu|eº W||||u +u e|oº|.e e|,||er+|ouº p|+que. A|||ouç| c|+ºº|||ed +rouç ||e |||º|e||uç d|º·
e+ºeº, |+r|||+| |eu|çu perp||çuº |+|d|, e.e| º|oWº |u|+c| .eº|c|eº +ud |º o||eu r|º|+|eu |o| |u|e||||ço.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 106
CIASSIFICAII0N
Two majoi types: pemphigus vulgaiis (PV)
and pemphigus foliaceus (PF). In addition,
paianeoplastic pemphigus (PP) associated with
malignancy and IgA pemphigus (Table 6-2).
£FI0£MI0I0C¥
PV: Raie, moie common in Jews and people of
Mediteiianean descent. In Jeiusalem the inci-
dence is estimated at 16 pei million, wheieas in
Fiance and Geimany it is 1.3 pei million.
PF: Also iaie but endemic in iuial aieas in
Biazil (fogo selvagem), wheie the pievalence
can be as high as 3.4%.
A¿e oI 0oset 40 to 60 yeais; fogo selvagem
also in childien and young adults.
Sex Equal incidence in males and females, but
piedominance of females with PF in Tunisia
and Colombia.
£II0I0C¥ AN0 FAIh0C£N£SIS
An autoimmune disoidei. Loss of the noimal
cell-to-cell adhesion in the epideimis ( atan-
ì|o|ysís ) occuis as a iesult of ciiculating anti-
bodies of the IgG class; these antibodies bind
to desmogleins, tiansmembiane glycopioteins
in the desmosomes, membeis of the cadheiin
supeifamily. In PV, desmoglein 3 (in some,
also desmoglein 1). All patients with PV have
autoantibodies to desmoglein 3. Those with
mucocutaneous PV also have antibodies to
desmoglein 1; those with only mucosal involve-
ment, only to desmoglein 3. In contiast, PF
patients have autoantibodies only to desmo-
glein 1. These autoantibodies inteifeie with
calcium-sensitive adhesion function and thus
induce acantholysis (Image 6-2).
A ºe||ouº, +cu|e o| c||ou|c, |u||ouº +u|o|rruue
d|ºe+ºe o| º||u +ud rucouº rer||+ueº |+ºed ou
+c+u||o|,º|º.
IWo r+jo| |,peº. perp||çuº .u|ç+||º (|\) +ud
perp||çuº |o||+ceuº (||)
|\. ||+cc|d |||º|e|º ou º||u +ud e|oº|ouº ou
rucouº rer||+ueº. ||. ºc+|, +ud c|uº|ed º||u
|eº|ouº.
|\. ºup|+|+º+| +c+u||o|,º|º. ||. ºu|co|ue+| +c+u·
||o|,º|º
|çC +u|o+u|||od|eº |o deºroç|e|uº, ||+uºrer·
||+ue deºroºor+| +d|eº|ou ro|ecu|eº.
'e||ouº +ud o||eu |+|+| uu|eºº ||e+|ed W||| |r·
ruuoºupp|eºº|.e +çeu|º.
F£MFhICüS |C|·9 . o9+.+

|C|·¹0 . |¹0
CIINICAI MANIF£SIAII0N
PV usually staits in the oial mucosa, and months
may elapse befoie skin lesions occui; lesions
may be localized foi months, aftei which genei-
alized bullae occui. Less fiequently theie may be
a geneialized, acute eiuption of bullae fiom the
beginning. No piuiitus (as occuis in pemphig-
oid but buining and pain in eiosions oi eioded
bullae). Painful and tendei mouth lesions may
pievent adequate food intake. Epistaxis, hoaise-
ness, dysphagia. Weakness, malaise, weight loss.
PF has no mucosal lesion and staits with scaly,
ciusted lesion on an eiythematous base, initially
in seboiiheic aieas.
Skìo Iesìoos oI Fv Round oi oval vesicles
and bullae with seious content, flaccid (flabby)
(Fig. 6-9), easily iuptuied, and weeping (Fig.
6-10), aiising on norma| skin, iandomly
scatteied, disciete. Localized (e.g., to mouth oi
ciicumsciibed skin aiea), oi geneialized with a
iandom pattein. Extensive eiosions that bleed
easily (Fig. 6-11), ciusts paiticulaily on scalp.
IA8I£ 6-2 C|assification of Pemphi¿us
|erp||çuº .u|ç+||º
|erp||çuº .u|ç+||º. |oc+||/ed +ud çeue|+||/ed
|erp||çuº .eçe|+uº. |oc+||/ed
||uç·|uduced
|erp||çuº |o||+ceuº
|erp||çuº |o||+ceuº. çeue|+||/ed
|erp||çuº e|,||er+|oºuº. |oc+||/ed
|oço ºe|.+çer. euder|c
||uç·|uduced
|+|+ueop|+º||c perp||çuº. +ººoc|+|ed W||| r+||çu+uc,
|çA perp||çuº. ºu|co|ue+| puº|u|+| de|r+|oº|º +ud
|u||+ep|de|r+| ueu||op||||c |çA de|r+||||º
S£CII0N 6 Bu||0u' ||'EA'E' 10T
FICük£ 6-9 Femphì¿us vu|¿arìs I||º |º ||e c|+ºº|c |u|||+| |eº|ou. ||+cc|d, e+º||, |up|u|ed .eº|c|e o| |u||+ ou
uo|r+|·+ppe+||uç º||u. kup|u|ed .eº|c|eº |e+d |o e|oº|ouº ||+| ºu|ºequeu||, c|uº|.
IMAC£ 6-2 0esmo¿|eìo compeosatìoo I||+uç|eº |ep|eºeu| ||e d|º||||u||ou o| |ºç ¹ +ud ! |u º||u +ud
rucouº rer||+ueº. Au||·|ºç ¹ +u|||od|eº |u perp||çuº |o||+ceuº c+uºe +c+u||o|,º|º ou|, |u ||e ºupe|||c|+| ep|·
de|r|º o| º||u. |u ||e deep ep|de|r|º +ud |u rucouº rer||+ueº, |ºç ! corpeuº+|eº |o| +u|||od,·|uduced |oºº
o| |uuc||ou o| |ºç ¹. |u e+||, perp||çuº .u|ç+||º, +u|||od|eº +|e p|eºeu| ou|, +ç+|uº| |ºç !, W||c| c+uºe |||º|e|º
ou|, |u ||e deep rucouº rer||+ue W|e|e |ºç ! |º p|eºeu| W|||ou| corpeuº+|o|, |ºç ¹. noWe.e|, |u ruco·
cu|+ueouº perp||çuº, +u|||od|eº +ç+|uº| |o|| |ºç ¹ +ud |ºç ! +|e p|eºeu|, +ud |||º|e|º |o|r |u |o|| rucouº
rer||+ue +ud º||u. I|e |||º|e| |º deep p|o|+||, |ec+uºe +u|||od|eº d|||uºe ||or ||e de|r|º +ud |u|e||e|e |||º|
W||| ||e |uuc||ou o| deºroºoreº +| ||e |+ºe o| ||e ep|de|r|º. ¦||or l '|+u|e,, |u K wo||| e| +| (edº). |·|c¤c|··:|´º
|-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, 1|| ed. |eW \o||, \cC|+W·n|||, 2003, p +o!.|
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 108
Since blisteis iuptuie so easily, only eiosions aie
seen in many patients. These aie veiy painful
(Fig. 6-11).
NI|v|s|y SIgn Dislodging of epideimis by
lateial fingei piessuie in the vicinity of lesions,
which leads to an eiosion. Piessuie on bulla
leads to lateial extension of blistei.
SItes v] PredI|ectIvn Scalp, face, chest, axillae,
gioin, umbilicus. In bediidden patients, theie is
extensive involvement of back (Fig. 6-11).
Mucous Membraoes Bullae iaiely seen, eio-
sions of mouth and nose, phaiynx and laiynx,
vagina.
Skìo Iesìoos oI FF Most commonly on face,
scalp, uppei chest, and abdomen. Scaly, ciusted
eiosions on an eiythematous base. In eaily
oi localized disease, shaiply demaicated in
seboiiheic aieas; may stay localized foi a long
time oi piogiess to geneialized disease and
exfoliative eiythiodeima. Initial lesion also
a flaccid bulla but this is iaiely seen because of
supeificial location (see histopathology below).
vAkIANIS (S££ IA8I£ 6-2)
PemphIgus Vegetuns (PVeg) Usually confined
to inteitiiginous iegions, peiioial aiea, neck,
and scalp. Gianulomatous vegetating puiulent
plaques that extend centiifugally. In these pa-
tients theie is a gianulomatous iesponse to the
autoimmune damage of PV.
Drug-1nduced PV Clinically identical to spoi-
adic PV. Seveial diffeient diugs implicated, most
significantly, captopiil and D-penicillamine.
BruzI|Iun PemphIgus (Fvgv Se|vugem) A
distinctive foim of PF endemic to south cen-
tial Biazil. Clinically, histologically, and immu-
nopathologically identical to PF. Patients
impiove when moved to uiban aieas but ielapse
aftei ietuining to endemic iegions. It is specu-
lated that the disease is somehow ielated to an
aithiopod-boine infectious agent, with clustei-
ing similai to that of the ||at| [|y-Símí|íum
íntrímanum . Moie than 1000 new cases pei yeai
aie estimated to occui in the endemic iegions.
PemphIgus Erythemutvsus (PE) Synonym :
Seneai-Ushei syndiome. A localized vaii-
ety of PF laigely confined to seboiiheic sites.
Eiythematous, ciusted, and eiosive lesions in the
°butteifly" aiea of the face, foiehead, and piest-
einal and inteiscapulai iegions. These patients
have immunoglobulin and complement depos-
its at the deimal-epideimal junction, in addi-
tion to inteicellulai pemphigus antibody in the
epideimis, and may have antinucleai antibodies,
as is the case in lupus eiythematosus.
Drug-1nduced PemphIgus PF As in PV, associ-
ated with D-penicillamine and less fiequently
by captopiil and othei diugs. In most, but not
all, instances the eiuption iesolves aftei teimi-
nation of theiapy with the offending diug.
FAkAN£0FIASIIC F£MFhICüS (FNF)
This is a disease sui geneiis (see Section 18).
Mucous membianes piimaiily and most se-
veiely involved. Lesions combine featuies of
pemphigus vulgaiis and eiythema multifoime,
clinically and histologically.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y PV: Light micioscopy (se-
lect eaily small bulla oi, if not piesent, maigin
of laigei bulla oi eiosion): Sepaiation of keiati-
nocytes, supiabasally, leading to split just a|o·e
the basal cell layei and vesicles containing sepa-
iated, iounded-up (acantholytic) keiatinocytes.
PF: Supeificial foim with acantholysis in the
gianulai layei of the epideimis.
Immuoopatho|o¿y Diiect immunofluoies-
cence (IF) staining ieveals IgG and often C3
deposited in lesional and paialesional skin in
ì|e ínìerte||u|ar su|sìante o[ ì|e e¡íJermís .
Serum Autoantibodies (IgG) detected by indi-
iect IF (IIF) oi enzyme-linked immunosoibent
assay (ELISA). Titei usually coiielates with
activity of disease piocess. In PV autoantibod-
ies aie diiected against a 130-kDa glycopio-
tein designated desmoglein 3 and located in
desmosomes. In PF ciiculating autoantibodies
to a 160-kDa inteicellulai (cell suiface) antigen,
desmoglein 1, in the desmosomes of keiatino-
cytes. PV (130 kDa) and PF (160 kDa) antigens
diffei (Image 6-2). This explains the diffeient
sites of acantholysis and thus the diffeient clini-
cal appeaiance of the two conditions.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Can be a difficult pioblem if only mouth lesions
aie piesent. Aphthae, mucosal lichen planus,
eiythema multifoime. Diffeiential diagnosis
includes all foims of acquiied bullous diseases
(see Table 6-3). Biopsy of the skin and mucous
membiane, diiect IF, and demonstiation of
ciiculating autoantibodies confiim a high index
of suspicion.
C0ükS£
In most cases the disease inexoiably piogiess-
es to death unless tieated aggiessively with
S£CII0N 6 Bu||0u' ||'EA'E' 109
FICük£ 6-10 Femphì¿us vu|¿arìs w|deºp|e+d cou||ueu| ||+cc|d |||º|e|º ou ||e |oWe| |+c| o| + +0·,e+|·o|d
r+|e W|o |+d + çeue|+||/ed e|up||ou |uc|ud|uç ºc+|p +ud rucouº rer||+ueº. I|e e|oded |eº|ouº +|e e\||ere|,
p+|u|u|.
FICük£ 6-11 Femphì¿us vu|¿arìs w|deºp|e+d cou||ueu| e|oº|ouº ||+| +|e .e|, p+|u|u| +ud ||eed e+º||,
|u + 5!·,e+|·o|d r+|e. I|e|e +|e |+|d|, +u, |u|+c| |||º|e|º |ec+uºe ||e, +|e ºo ||+ç||e +ud ||e+| e+º||,. I|e ||ood
||+c|º ço º|deW+,º |ec+uºe ||e p+||eu| |+d |eeu |,|uç ou ||º ||ç|| º|de |e|o|e ||e p|o|oç|+p| W+º |+|eu.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 110
immunosuppiessive agents. The moitality iate
has been maikedly ieduced since tieatment has
become available. Cuiiently, moibidity ielated
to glucocoiticoids and immunosuppiesive
theiapies.
MANAC£M£NI
C|ucocortìcoìds 2 to 3 mg/kg body weight
of piednisone until cessation of new blistei
foimation and disappeaiance of Nikolsky sign.
Then iapid ieduction to about half the initial
dose until patient is almost cleai, followed by
veiy slow tapeiing of dose to minimal effective
maintenance dose.
Coocomìtaot Immuoosuppressìve Iherapy
Immunosuppiessive agents aie given concomi-
tantly foi theii glucocoiticoid-spaiing effect:
À:aì|ío¡ríne , 2-3 mg/kg body weight until
complete cleaiing; tapeiing of dose to
1 mg/kg. Azathiopiine alone is contin-
ued even aftei cessation of glucocoiticoid
tieatment and may have to be continued
foi many months oi yeais.
Meì|oìrexaìe , eithei oially (PO) oi IM at
doses of 25 to 35 mg/week. Dose adjust-
ments aie made as with azathiopiine.
Cyt|o¡|os¡|amíJe , 100-200 mg daily, with
ieduction to maintenance doses of 50-
100 mg/d. Alteinatively, cyclophospha-
mide °bolus" theiapy with 1000 mg IV
once a week oi eveiy 2 weeks in the
initial phases, followed by 50-100 mg/d
PO as maintenance.
P|asma¡|eresís , in conjunction with glu-
cocoiticoids and immunosuppiessive
agents in pooily contiolled patients, in
the initial phases of tieatment to ieduce
antibody titeis.
Co|J ì|era¡y , foi mildei cases. Aftei an
initial test dose of 10 mg IM, 25-50 mg
of gold sodium thiomalate is given IM at
weekly inteivals to a maximum cumula-
tive dose of 1 g.
Myto¡|eno|aìe mo[eìí| (1 g twice daily) has
been iepoited to be beneficial, and clini-
cal studies aie ongoing.
Híg|-Jose ínìra·enous ímmunog|o|u|ín
(HIVIg) (2 g/kg body weight eveiy 3-4
weeks) has been iepoited to have a glu-
cocoiticoid-spaiing effect. Expensive.
Ríìuxíma| (monoclonal antibody to CD20)
piesumably taigets B cells, the piecuisois
of (auto) antibody-pioducing plasma
cells. Given as intiavenous theiapy once a
week foi 4 weeks, shows diamatic effects
in some and at least paitial iemission in
othei patients. Seiious infections may
be seen.
0ther Measures Cleansing baths, wet diess-
ings, topical and intialesional glucocoiticoids,
antimiciobial theiapy pei documented bacte-
iial infections. Coiiection of fluid and electio-
lyte imbalance.
Mooìtorìo¿ Clinical, foi impiovement of
skin lesions and development of diug-ielated
side effects. Laboiatoiy monitoiing of pem-
phigus antibody titeis and foi hematologic
and metabolic indicatois of glucocoiticoid-
and/oi immunosuppiessive-induced adveise
effects.
S£CII0N 6 Bu||0u' ||'EA'E' 111
IA8I£ 6-3 0ifferentia| 0ia¿nosis of |mportant Acquired Bu||ous 0iseases
0ìsease Skìo Iesìoos Mucous Membraoes 0ìstrìbutìoo
|\ ||+cc|d |u||+e ou uo|r+| A|roº| +|W+,º |u.o|.ed, Au,W|e|e, |oc+||/ed o|
º||u, e|oº|ouº e|oº|ouº çeue|+||/ed
|| C|uº|ed e|oº|ouº, occ+º|ou+||, k+|e|, |u.o|.ed E\poºed, ºe|o|||e|c
||+cc|d .eº|c|eº |eç|ouº o| çeue|+||/ed
|\eç C|+uu|+||uç p|+queº, Aº |u |\ |u|e||||ç|uouº |eç|ouº, ºc+|p
occ+º|ou+||, .eº|c|eº +|
r+|ç|u
Bu||ouº Ieuºe |u||+e ou uo|r+| \ou|| |u.o|.ed |u Au,W|e|e, |oc+||/ed o|
perp||ço|d +ud e|,||er+|ouº ¹0-!5° çeue|+||/ed
º||u, u|||c+||+| p|+queº
+ud p+pu|eº
EBA Ieuºe |u||+e +ud e|oº|ouº, \+, |e ºe.e|e|, I|+ur+||/ed |eç|ouº o|
uou|u||+rr+|o|, o| B|·, |u.o|.ed (o|+| |+udor
|n· o| |A|·|||e p|eºeu|+||ou eºop|+çuº, .+ç|u+)
|e|r+||||º C|ouped p+pu|eº, .eº|c|eº, |oue ||ed||ec||ou º||eº. e||oWº,
|e|pe|||o|r|º u|||c+||+| p|+queº, c|uº|ed |ueeº, ç|u|e+|, º+c|+|, +ud
ºc+pu|+| +|e+º
||ue+| |çA Auuu|+|, ç|ouped p+pu|eº, 0|+| e|oº|ouº +ud u|ce|º, Au,W|e|e
de|r+|oº|º .eº|c|eº, +ud |u||+e coujuuc||.+| e|oº|ouº
+ud ºc+|||uç
0ìsease hìstopatho|o¿y Immuoopatho|o¿y[Skìo Serum
|\ 'up|+|+º+| +c+u||o|,º|º |çC |u|e|ce||u|+| p+||e|u |çC AB |o |u|e|ce||u|+|
ºu|º|+uce o| ep|de|r|º (|||)
E||'A. AB |o deºroç|e|u
! >> deºroç|e|u ¹
|| Ac+u||o|,º|º |u ç|+uu|+| |çC, |u||+ce||u|+| p+||e|u |çC AB |o |u|e|ce||u|+|
|+,e| ºu|º|+uce o| ep|de|r|º (|||)
E||'A. AB |o deºroç|e|u
¹ ou|,
|\eç Ac+u||o|,º|º ± |u||+ep|de|r+| Aº |u |\ Aº |u |\
ueu||op||||c +|ºceººeº,
ep|de|r+| |,pe|p|+º|+
Bu||ouº 'u|ep|de|r+| |||º|e| |çC +ud C! ||ue+| +| |çC AB |o B\/ (|||),
perp||ço|d B\/ d||ec|ed |o B|AC¹ +ud
B|AC2
EBA 'u|ep|de|r+| |||º|e| ||ue+| |çC +| B\/ |çC AB |o B\/ (|||) d||ec|ed
|o |,pe \|| co||+çeu (E||'A,
weº|e|u ||o|)
|e|r+||||º |+p|||+|, r|c|o+|ºceººeº, C|+uu|+| |çA |u ||pº o| Au||eudor,º|+|
|e|pe|||o|r|º ºu|ep|de|r+| .eº|c|e p+p|||+e +u|||od|eº
||ue+| |çA 'u|ep|de|r+| |||º|e| ||ue+| |çA +| B\/ |oW |||e|º o| |çA AB
de|r+|oº|º W||| ueu||op|||º +ç+|uº| B\/
|o|e. AB, +u|||od,, B\/, |+ºereu| rer||+ue /oue, B|, |u||ouº perp||ço|d, |n, de|r+||||º |e|pe|||o|r|º, EB, ep|de|ro|,º|º |u||oº+ +cqu|º||+,
E||'A, eu/,re·||u|ed |rruuoºo||eu| +ºº+,, |||, |ud||ec| |rruuo||uo|eºceuce, |A|, ||ue+| |çA de|r+|oº|º, ||, perp||çuº |o||+ceuº, |\, perp||·
çuº .u|ç+||º, |\eç, perp||çuº .eçe|+uº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 112
£FI0£MI0I0C¥
A¿e oI 0oset 60 to 80 yeais.
Sex Equal incidence in males and females. No
known iacial piedilection.
Iocìdeoce The most common bullous autoim-
mune disease. Seven pei million in Geimany
and Fiance. Fai moie common in authois`
expeiience in veiy old people.
£II0I0C¥ AN0 FAIh0C£N£SIS
Inteiaction of autoantibody with bullous pem-
phigoid antigen ¦BPAG1 and BPAG2 (collagen
type XVII)] in hemidesmosomes of basal ke-
iatinocytes (Image 6-1) is followed by comple-
ment activation and attiaction of neutiophils
and eosinophils. Bullous lesion iesults fiom
inteiaction of multiple bioactive molecules ie-
leased fiom inflammatoiy cells. Not yet com-
pletely claiified.
CIINICAI MANIF£SIAII0N
Often staits with a piodiomal eiuption (ui-
ticaiial, papulai lesions) and evolves in weeks
to months to bullae that may appeai sud-
denly as a geneialized eiuption. Initially no
symptoms except modeiate oi seveie piuiitus;
latei, tendeiness of eioded lesions. No constitu-
tional symptoms, except in widespiead, seveie
disease.
Skìo Iesìoos Eiythematous, papulai oi
uiticaiial-type lesions (Fig. 6-12) may piecede
bullae foimation by months. Bullae: laige, tense,
fiim-topped, oval oi iound (Fig. 6-13); may
aiise in noimal, eiythematous, oi uiticaiial skin
and contain seious oi hemoiihagic fluid. The
eiuption may be localized oi geneialized, usually
scatteied but also giouped in aicifoim and
seipiginous patteins. Bullae iuptuie less easily
than in pemphigus, but sometimes laige, biight
ied, oozing, and bleeding eiosions become a
A |u||ouº +u|o|rruue d|ºe+ºe uºu+||, |u e|de||,
p+||eu|º.
||u||||c p+pu|+| +ud/o| u|||c+||+| |eº|ouº W||| |+|çe
|euºe |u||+e.
'u|ep|de|r+| |||º|e|º W||| eoº|uop|||º.
C! +ud |çC +| ep|de|r+| |+ºereu| rer||+ue, +u||·
|+ºereu| rer||+ue |çC +u|o+u|||od|eº |u ºe|ur.
Au|o+u||çeuº +|e |e|+||uoc,|e |er|deºroºore
p|o|e|uº.
I|e|+p, |uc|udeº |op|c+| +ud º,º|er|c ç|ucoco|||·
co|dº +ud o||e| |rruuoºupp|eºº|.eº.
8üII0üS F£MFhIC0I0 (8F) |C|·9 . o9+.5

|C|·¹0 . |¹2.0
majoi pioblem. Usually, howevei, the oiiginally
tense bullae collapse and tiansfoim into ciusts.
SItes v] PredI|ectIvn Axillae; medial aspects of
thighs, gioins, abdomen; flexoi aspects of foie-
aims; lowei legs (often fiist manifestation).
Mucous Membraoes Piactically only in the
mouth (10-35%); less seveie and painful and
less easily iuptuied than in pemphigus.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y LIght MIcrvscvpy Neu-
tiophils in °Indian-file" alignment at deimal-
epideimal junction; neutiophils, eosinophils,
and lymphocytes in papillaiy deimis; su|e¡í-
Jerma| bulla.
E|ectrvn MIcrvscvpy Junctional cleavage, i.e.,
split occuis in lamina lucida of basement mem-
biane.
Immuoopatho|o¿y Lineai IgG deposits along
the basement membiane zone. Also, C3, which
may occui in the absence of IgG.
Serum Ciiculating antibasement membiane
IgG antibodies detected by IIF in 70% of pa-
tients. Titeis do not coiielate with couise of
disease. Autoantibodies in bullous pemphigoid
iecognize two types of antigens. BPAG1 is a
230-kDa glycopiotein that has high homology
with desmoplakin I and is pait of hemidesmo-
somes. BPAG2 is a tiansmembianous 180-kDa
polypeptide (type XVII collagen).
hemato|o¿y Eosinophilia (not always).
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical appeaiance, histopathology, and immu-
nology peimit a diffeientiation fiom othei bul-
lous diseases (see Table 6-3 on p 111).
MANAC£M£NI
Systemic piednisone with staiting doses of
50-100 mg/d continued until cleai, eithei alone
S£CII0N 6 Bu||0u' ||'EA'E' 113
oi combined with azathiopiine, 150 mg daily,
foi iemission induction and 50-100 mg foi
maintenance; in iefiactoiy cases IVIG: plas-
mapheiesis in mildei cases, sulfones (dapsone),
100-150 mg/d. Low-dose MTX 2.5 to 10 mg
weekly PO is effective and safe in eldeily. In veiy
mild cases and foi local iecuiiences, topical glu-
cocoiticoid oi topical taciolimus theiapy may
be beneficial. Tetiacycline ± nicotinamide has
been iepoited to be effective in some cases.
C0ükS£ AN0 Fk0CN0SIS
Patients often go into a peimanent iemission
aftei theiapy and do not iequiie fuithei theiapy;
local iecuiiences can sometimes be contiolled
with topical glucocoiticoids; clobetasol with
occlusion to uiticaiial aieas. Also, intialesional
tiiamcinolone foi localized disease. Some
cases go into spontaneous iemission without
theiapy.
FICük£ 6-12 8u||ous pemphì¿oìd E+||, |eº|ouº |u + 15·,e+|·o|d |er+|e. |o|e u|||c+||+| p|+queº +ud +
ºr+||, |euºe |||º|e| W||| + c|e+| ºe|ouº cou|eu|.
FICük£ 6-13 8u||ous pemphì¿oìd I||º 11·,e+|·o|d r+|e |+º + çeue|+||/ed e|up||ou W||| cou||ueu| u|||·
c+||+| p|+queº +ud ru|||p|e |euºe |||º|e|º. I|e coud|||ou |º ºe.e|e|, p|u||||c.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 114
A |+|e d|ºe+ºe, |+|çe|, o| ||e e|de||,.
B||º|e|º ||+| |up|u|e e+º||, +ud +|ºo e|oº|ouº
|eºu|||uç ||or ep|||e||+| ||+ç||||, |u ||e rou||,
o|op|+|,u\, +ud, ro|e |+|e|,, ||e u+ºop|+|,u·
çe+|, eºop|+çe+|, çeu||+|, +ud |ec|+| rucoº+e.
0cu|+| |u.o|.ereu| r+, |u|||+||, r+u||eº| +º
uu||+|e|+| o| |||+|e|+| coujuuc||.|||º W||| |u|u|uç,
d|,ueºº, +ud |o|e|çu·|od, ºeuº+||ou.
C||ou|c |u.o|.ereu| |eºu||º |u ºc+|||uç, º,r·
||ep|+|ou (||ç. o·¹+), +ud, |u ºe.e|e d|ºe+ºe,
|uº|ou o| ||e |u||+| +ud p+|pe||+| coujuuc||.+.
Eu||op|ou +ud |||c||+º|º |eºu|| |u co|ue+| |||||+·
||ou, ºupe|||c|+| puuc|+|e |e|+||uop+||,, co|ue+|
ueo.+ºcu|+||/+||ou, u|ce|+||ou, +ud |||udueºº.
'c+|||uç +|ºo |u ||e |+|,u\, eºop|+çe+| |u.o|.e·
reu| |eºu||º |u º|||c|u|e |o|r+||ou |e+d|uç |o
d,ºp|+ç|+ o| d,uop|+ç|+.
I|e º||u |º |u.o|.ed |u |ouç||, !0° o| p+||eu|º.
Au||çeuº |o W||c| +u|o+u|||od|eº r+, |e d||ec|ed
|uc|ude B|AC2, |+r|u|u 5, |u|eç||u ºu|uu||º
+
+ud
o
, |,pe \|| co||+çeu, +ud B|AC¹.
3·±¤º|·¤¸·|-··, ¤-¤¤|·¸¤·! deºc|||eº + ºu|ºe|
o| p+||eu|º W|oºe º||u |eº|ouº |ecu| +| ||e º+re
º||eº, r+|u|, ou ||e |e+d +ud uec|, +ud +|ºo |e+d
|o ºc+|||uç.
!c¤c¸-¤-¤|. \oº| p+||eu|º |eºpoud |o d+pºoue
|u cor||u+||ou W||| |oW·doºe p|edu|ºoue. 'ore
p+||eu|º r+, |equ||e ro|e +çç|eºº|.e |rruuo·
ºupp|eºº|.e ||e+|reu| W||| c,c|op|oºp|+r|de o|
+/+|||op||ue, |u cor||u+||ou W||| ç|ucoco|||co|dº.
|u +dd|||ou, ºu|ç|c+| |u|e|.eu||ou |o| ºc+|||uç +ud
ºuppo|||.e re+ºu|eº.
',¤¤¤,¤. \ucouº rer||+ue perp||ço|d.
CICAIkICIAI F£MFhIC0I0 (CF) |C|·9 . o9+.o

|C|·¹0 . |¹2.¹
FICük£ 6-14 Cìcatrìcìa| pemphì¿oìd I||º ºc+|||uç coud|||ou |u + 13·,e+|·o|d |er+|e º|+||ed W||| |||+|e|+|
coujuuc||.+| p+|u +ud |o|e|çu |od, ºeuº+||ou +º ||e |||º| º,rp|orº. I|e coujuuc||.+ ||eu |ec+re e|oº|.e W|||
ºc+|||uç +ud ||||ouº ||+c|º |e|Weeu e,e||dº +ud ||e e,e.
S£CII0N 6 Bu||0u' ||'EA'E' 115
A |+|e p|u||||c +ud po|,ro|p||c |u||+rr+|o|, |u|·
|ouº de|r+|oº|º o| p|eçu+uc, +ud ||e poº|p+||ur
pe||od.
I|e eº||r+|ed |uc|deuce |º ||or ¹ |u ¹100 |o ¹ |u
¹0,000 de||.e||eº.
E\||ere|, p|u||||c .eº|cu|+| e|up||ou r+|u|, ou
||e +|doreu |u| +|ºo ou o||e| +|e+º, W||| ºp+|·
|uç o| ||e rucouº rer||+ueº. |eº|ouº .+|, ||or
e|,||er+|ouº, eder+|ouº p+pu|eº |o u|||c+||+|
p|+queº |o .eº|c|eº +ud |euºe |u||+e (||ç. o·¹5).
|C uºu+||, |eç|uº ||or ||e |ou||| |o ||e ºe.eu||
rou|| o| p|eçu+uc, |u| c+u +|ºo occu| |u ||e
|||º| |||reº|e| +ud |u ||e |rred|+|e poº|p+||ur
pe||od.
|| r+, |ecu| |u ºu|ºequeu| p|eçu+uc|eº, || || doeº,
|| |º |||e|, |o |eç|u e+|||e|.
|C c+u |e e\+ce||+|ed |, ||e uºe o| eº||oçeu·
+ud p|oçeº|e|oue·cou|+|u|uç red|c+||ouº.
n|º|op+||o|oç|c+||, || |º + ºu|ep|de|r+| |||º|e||uç
coud|||ou, +ud ||e|e |º + |e+., ||ue+| depoº|||ou
o| C! +|ouç ||e |+ºereu| rer||+ue /oue W|||
coucor||+u| |çC depoº|||ou |u |ouç||, !0° o|
p+||eu|º.
'e|ur cou|+|uº |çC +u|||+º+| rer||+ue +u|||od·
|eº, |u| ||eºe +|e de|ec|ed |u ou|, 20° o| p+||eu|º
|, |||. E||'A +ud |rruuo||o|||uç +ºº+,º de|ec|
+u|o+u|||od|eº |u >10°, d||ec|ed |o B|¹30 (|,pe
/\|| co||+çeu), + ¹30·||+ ||+uºrer||+ue p|o|e|u
|u |er|deºroºoreº. I|e, +|e +.|d corp|ereu|·
||\|uç |çC¹ +u|||od|eº ||+| ||ud |o +ru|o||c
ep|||e||+| |+ºereu| rer||+ue. I|e, c+u +|ºo |e
de|ec|ed |u ||e ||ood o| ºore |u|+u|º.
'ore 5° o| |+||eº |o|u |o ro||e|º W||| |C
|+.e u|||c+||+|, .eº|cu|+|, o| |u||ouº |eº|ouº, W||c|
|eºo|.e ºpou|+ueouº|, du||uç ||e |||º| ºe.e|+|
Wee|º. |o º|çu|||c+u| r+|e|u+| ro|||d||, (p|u·
|||uº) +ud ro||+|||,. I|e|e |º + º||ç|| |uc|e+ºe |u
p|er+|u|e +ud ºr+||·|o|·çeº|+||ou+|·+çe |||||º.
'ore |epo||º o| |e|+| p|oçuoº|º |+.e |e.e+|ed
º|çu|||c+u| |e|+| de+|| +ud p|er+|u|e de||.e||eº,
W|e|e+º o||e|º |+.e ºuççeº|ed uo |uc|e+ºe |u
|e|+| ro||+|||,.
!c¤c¸-¤-¤| |º çe+|ed |o ºupp|eºº|uç |||º|e|
|o|r+||ou +ud |e||e.|uç ||e |u|euºe p|u|||uº.
||edu|ºoue, 20-+0 rç/d, |º ç|.eu |u| ºore||reº
||ç|e| doºeº +|e |equ||ed. ||edu|ºoue |º |+pe|ed
ç|+du+||, du||uç ||e poº|p+||ur pe||od. 0u|, +
|eW p+||eu|º do uo| |equ||e º,º|er|c p|edu|ºoue
+ud c+u |e r+u+çed W||| +u||||º|+r|ueº +ud
|op|c+| ç|ucoco|||co|dº.
F£MFhIC0I0 C£SIAII0NIS (FC) |C|·9 . o+o.3

|C|·¹0 . |¹2.3
FICük£ 6-15 Femphì¿oìd
¿estatìooìs  E|,||er+|ouº
p+pu|eº ||+| We|e ||ç||, p|u||||c
+ud |+d +ppe+|ed ou ||e ||uu|
+ud +|doreu o| |||º !!·,e+|·o|d
p|eçu+u| |er+|e (||||d |||reº|e|)
+ud We|e + c+uºe o| ç|e+| cou·
ce|u. A| |||º ||re ||e|e We|e uo
|||º|e|º +ud d|+çuoº|º W+º eº|+|·
||º|ed |, ||opº, +ud
|rruuop+||o|oç,.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 116
A c||ou|c, |ecu||eu|, |u|euºe|, p|u||||c e|up||ou
occu|||uç º,rre|||c+||, ou ||e e\||er|||eº +ud
||e ||uu|.
Couº|º|º o| ||u, .eº|c|eº, p+pu|eº, +ud u|||c+||+|
p|+queº ||+| +|e +||+uçed |u ç|oupº.
Aººoc|+|ed W||| ç|u|eu·ºeuº|||.e eu|e|op+||,
(C'E).
C|+|+c|e||/ed ||º|o|oç|c+||, |, p+p|||+|, co||ec||ou
o| ueu||op|||º.
C|+uu|+| |çA depoº||º |u p+|+|eº|ou+| o| uo|r+|
º||u +|e d|+çuoº||c.
keºpoudº |o ºu||+ d|uçº +ud, |o + |eººe| e\|eu|, |o
+ ç|u|eu·||ee d|e|.
0£kMAIIIIS h£kF£IIF0kMIS (0h) |C|·9 . o9+.0

|C|·¹0 . |¹!.0
£FI0£MI0I0C¥
Pievalence in Caucasians vaiies fiom 10 to 39
pei 100,000 peisons.
A¿e oI 0oset 20 to 60 yeais, but most com-
mon at 30 to 40 yeais; may occui in childien.
Sex Male:female iatio is 2:1.
£II0I0C¥ AN0 FAIh0C£N£SIS
The GSE piobably ielates to IgA deposits in the
skin. Patients have antibodies to tiansglutami-
nases (TGs) that may be the majoi autoantigens
in this disease. Epideimal TG autoantibody
piobably binds to TG in the gut and ciiculates
eithei alone oi as immune complexes and
deposits in skin. With additional factois IgA
activates complement via the alteinative path-
way, with subsequent chemotaxis of neutiophils
ieleasing theii enzymes and pioducing tissue
injuiy.
CIINICAI MANIF£SIAII0N
Piuiitus, intense, episodic; buining oi sting-
ing of the skin; iaiely, piuiitus may be absent.
FICük£ 6-15 (Cou||uued)
 u|||c+||+| p|+queº +ud ºr+|| .eºc|c|eº +ud |||º|e|º |u +uo||e| p+||eu| W|o |+d º|r||+| e|up||ouº |u p|e.|ouº
p|eçu+uc|eº. '|e |eºpouded |+p|d|, |o º,º|er|c ç|ucoco|||co|dº. I|e de||.e|, W+º uue.eu||u|, +ud ||e |+|, W+º
|e+|||,.
S£CII0N 6 Bu||0u' ||'EA'E' 11T
Symptoms often piecede the appeaiance of skin
lesions by 8 to 12 h. Ingestion of iodides and
oveiload of gluten aie exaceibating factois.
Systems kevìew Laboiatoiy evidence of small-
bowel malabsoiption is detected in 10-20%.
GSE occuis in neaily all patients and is demon-
stiated by small-bowel biopsy. Theie aie usually
no systemic symptoms.
Skìo Iesìoos Lesions consist of eiythematous
papules oi wheal-like plaques; tiny fiim-topped
vesicles, sometimes hemoiihagic (Fig. 6-16);
occasionally bullae. Lesions aie aiianged in
gioups (hence the name |er¡erìí[ormís ); the
distiibution is stiikingly symmetiic. Sciatching
iesults in excoiiations, ciusts (Fig. 6-17).
Postinflammatoiy hypei-and hypopigmentation
at sites of healed lesions.
SItes v] PredI|ectIvn Typical and almost diag-
nostic: extensoi aieas-elbows, knees. Buttocks,
scapulai and sacial aieas (Image 6-3 and Figs.
6-16 and 6-17). Heie, often in a °butteifly"
fashion. Scalp, face, and haiiline.
IA80kAI0k¥ £XAMINAII0NS
Immuoo¿eoetìcs Association with HLA-B8,
HLA-DR, and HLA-DQ.
0ermatopatho|o¿y Biopsy is best fiom eaily
eiythematous papule. Micioabscesses (poly-
moiphonucleai cells and eosinophils) at the
tips of the deimal papillae. Deimal infiltiation
of neutiophils and eosinophils. Su|e¡íJerma|
·esít|e .
ImmuooI|uoresceoce Of ¡erí|esíona| skin, best
on the buttocks. Gianulai IgA deposits in tips
of papillae that coiielate well with small-bowel
disease. Gianulai IgA is found in almost-noimal
skin in most patients and is diagnostic. Also
found aie C3 and C5and alteinative comple-
ment pathway components.
Cìrcu|atìo¿ Autoaotìbodìes Antiieticulin anti-
bodies of the IgA and IgG types, thyioid antimi-
ciosomal antibodies, and antinucleai antibodies
can be piesent. Putative immune complexes in
20-40% of patients. IgA antibodies binding to
the inteimyofibiil substance of smooth muscles
(anìíenJomysía| anìí|oJíes ) aie piesent in most
patients and have specificity foi TGs.
0ther Studìes Steatoiihea (20-30%) and ab-
noimal D-xylose absoiption (10-73%). Anemia
secondaiy to iion oi folate deficiency. EnJos-
to¡y o[ sma|| |owe| : blunting and flattening
of the villi (80-90%) in the small bowel as in
celiac disease. Lesions aie focal; veiification is
by small-bowel biopsy.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Giouped papulovesicles at piedilection sites
accompanied by seveie piuiitus aie highly sug-
gestive. Biopsy of eaily lesions usually diagnostic,
but IgA deposits in peiilesional skin detected by
IF aie the best confiiming evidence. Diffeiential
FICük£ 6-16 0ermatìtìs herpetìIormìs I|eºe +|e ||e c|+ºº|c e+||, |eº|ouº. |+pu|eº, u|||c+||+| p|+queº, ºr+||
ç|ouped .eº|c|eº, +ud c|uº|º ou ||e e||oW o| + 2!·,e+|·o|d r+|e.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 118
diagnosis is to alleigic contact deimatitis, atopic
deimatitis, scabies, neuiotic excoiiations, papu-
lai uiticaiia, bullous pemphigoid, pemphigoid
gestationis (see Table 6-3).
C0ükS£
Piolonged, foi many yeais, with
a thiid of the patients eventually
having a spontaneous iemission.
MANAC£M£NI
Systemìc Iherapy Dupsvne
100-150 mg daily, with giadual
ieduction to 50-25 mg and often
as low as 50 mg twice a week.
Theie is a diamatic iesponse, often
within houis. Obtain a glucose-
6-phosphate dehydiogenase level
befoie staiting sulfones; obtain
methemoglobin levels in the initial
2 weeks, and follow blood counts
caiefully foi the fiist few months.
IMAC£ 6-3 |e|r+||||º |e|pe|||o|r|º.
|+||e|u o| d|º||||u||ou.
Su|]upyrIdIne 1-1.5 g/d, with plenty of fluids,
if dapsone contiaindicated oi not toleiated.
Monitoi foi casts in uiine and kidney function.
0ìet A gluten-fiee diet may suppiess the
disease oi allow ieduction of the dosage of dap-
sone oi sulfapyiidine, but iesponse is veiy slow.
FICük£ 6-1T 0ermatìtìs herpetìIormìs
|u |||º 5o·,e+|·o|d r+|e p+||eu| W||| +
çeue|+||/ed ||ç||, p|u||||c e|up||ou, ||e
d|+çuoº|º c+u |e r+de upou |||º| º|ç|| |,
||e d|º||||u||ou o| ||e |eº|ouº. \oº| |e+.·
||, |u.o|.ed +|e ||e e||oWº, ||e ºc+pu|+|,
º+c|+|, +ud ç|u|e+| +|e+º, +ud (uo| ºeeu
|u |||º p|c|u|e) ||e |ueeº. upou c|oºe
|uºpec||ou ||e|e +|e ç|ouped p+pu|eº,
ºr+|| .eº|c|eº, c|uº|º, +ud e|oº|ouº ou +u
e|,||er+|ouº |+ºe +ud ||e|e |º poº||u||+r·
r+|o|, |,po· +ud |,pe|p|çreu|+||ou.
Bec+uºe o| p|u|||uº ||e p+||eu| |+d p|e.|·
ouº|, |eeu d|+çuoºed +º |+.|uç +|op|c
de|r+||||º, ºc+||eº, +ud +||e|ç|c cou|+c|
de|r+||||º +ud |+d |eºpouded ou|, poo||,
|o |op|c+| ç|ucoco|||co|dº. I||º p+|||cu|+|
e|up||ou occu||ed +||e| |e |+d ºpeu| +
.+c+||ou ou ||e |+|r+||ou co+º| (|+.·
|uç |eeu |o|d ||+| ºuu|+|||uç Wou|d |e
çood |o| ||º coud|||ou) W|e|e ||º re+|º
couº|º|ed o| ºe+|ood (|od|deº) +ud W|||e
||e+d (ç|u|eu).
S£CII0N 6 Bu||0u' ||'EA'E' 119
A |+|e, |rruue·red|+|ed, ºu|ep|de|r+| |||º|e|·
|uç º||u d|ºe+ºe de||ued |, ||e p|eºeuce o| |oro·
çeueouº ||ue+| depoº||º o| |çA +| ||e cu|+ueouº
|+ºereu| rer||+ue /oue (|r+çe o·¹).
|| |º c|e+||, ºep+|+|e ||or de|r+||||º |e|pe|||o|r|º
(|n) ou ||e |+º|º o| |rruuop+||o|oç,, |rruuo·
çeue||cº, +ud |+c| o| +ººoc|+||ou W||| C'E.
|A| roº| o||eu occu|º +||e| pu|e||,.
C||u|c+| r+u||eº|+||ouº +|e .e|, º|r||+| |o ||oºe o|
|n, |u| ||e|e |º ro|e |||º|e||uç. |+||eu|º p|eºeu|
W||| cor||u+||ouº o| +uuu|+| o| ç|ouped p+pu|eº,
.eº|c|eº, +ud |u||+e (||ç. o·¹3) ||+| +|e d|º||||u|ed
º,rre|||c+||, ou ||uu| +ud e\||er|||eº |uc|ud|uç
e||oWº, |ueeº, +ud |u||oc|º. I|e |eº|ouº +|e .e|,
p|u||||c |u| |eºº ºe.e|e ||+u ||oºe o| |n.
\ucoº+| |u.o|.ereu| |º |rpo||+u| +ud |+uçeº
||or |+|çe +º,rp|or+||c o|+| e|oº|ouº +ud u|ce|·
+||ou |o ºe.e|e o|+| d|ºe+ºe +|oue, o| ºe.e|e çeu·
e|+||/ed cu|+ueouº |u.o|.ereu| +ud o|+| d|ºe+ºe
º|r||+| |o ||+| |u c|c+|||c|+| perp||ço|d.
|| |º |deu||c+| W||| c||ou|c |u||ouº d|ºe+ºe o| c|||d·
|ood (CB|C), W||c| |º + |+|e |||º|e||uç d|ºe+ºe
||+| occu|º p|edor|u+u||, |u c|||d|eu ·5 ,e+|º
(||ç. o·¹9).
C||cu|+||uç +u|o+u|||od|eº +ç+|uº| .+||ouº ep|de|·
r+| |+ºereu| rer||+ue +u||çeuº |+.e |eeu
|ouud.
|A| |+º |eeu +ººoc|+|ed W||| d|uçº. .+ucor,·
c|u, |||||ur, p|eu,|o|u, ºu||+re||o\+/o|e/|||r·
e||op||r, |u|oºer|de, c+p|op|||, d|c|o|eu+c, +ud
o||e|º.
I|e|e |º + ºr+|| ||º| o| |,rp|o|d r+||çu+uc|eº,
+ud +ººoc|+|ed u|ce|+||.e co||||º |+º |eeu |e·
po||ed.
!c¤c¸-¤-¤|. |+||eu|º |eºpoud |o d+pºoue o|
ºu||+p,||d|ue |u| |u +dd|||ou, roº| r+, |equ||e
|oW·doºe p|edu|ºoue. |+||eu|º do uo| |eºpoud |o
+ ç|u|eu·||ee d|e|.
IIN£Ak I¿A 0£kMAI0SIS (IA0) |C|·9 . 102.3
FICük£ 6-18 Iìoear I¿A dermatosìs I|e|e +|e
ru|||p|e ç|ouped, cou||ueu| .eº|c|eº, |u||+e, +ud c|uº|º
ou +u u|||c+||+| +ud e|,||er+|ouº |+ºe. I|e|e We|e
º|r||+| |eº|ouº ou ||e ||uu| +ud ||e uppe| e\||er|||eº.
FICük£ 6-19 Iìoear I¿A dermatosìs (chrooìc,
bu||ous dìsease oI chì|dhood) E\|euº|.e |||º|e|·
|uç ou ||e uppe| e\||er|||eº +ud ||uu| |u + 1·,e+|·o|d
c|||d. |o|e. |||º|e|º +|e |o|| |euºe +ud ||+cc|d. I|e,
+|e ç|ouped +ud ||e|e |º uo uo|+||e |u||+rr+||ou.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 120
A c||ou|c ºu|ep|de|r+| |u||ouº d|ºe+ºe +ººoc|·
+|ed W||| +u|o|rruu||, |o ||e |,pe \|| co||+çeu
W||||u ||e +uc|o||uç ||||||º |u ||e |+ºereu|
rer||+ue /oue.
|ou| |,peº. ||e :|cºº·: ¤-:|c¤¤·|±||¤±º ¤·-º-¤·
|c|·¤¤ |º + uou|u||+rr+|o|,, |||º|e||uç e|up||ou
W||| +c|+| d|º||||u||ou ||+| |e+|º W||| ºc+|||uç +ud
r|||+ |o|r+||ou. || |º + rec|+uo·|u||ouº d|ºe+ºe
r+||ed |, º||u ||+ç||||,, +ud p+||eu|º |+.e |euºe
|||º|e|º W||||u uou|u||+red º||u, e|oº|ouº, +ud
ºc+|º |u ||+ur+||/ed |eç|ouº ºuc| +º ||e do|º+
o| ||e |+udº, |uuc||eº, e||oWº, |ueeº, º+c|+|
+|e+, +ud |oeº. I||º p|eºeu|+||ou ||uº |eºer||eº
po|p|,||+ cu|+ue+ |+|d+ (ºee 'ec||ou ¹0) o| |e·
|ed||+|, ep|de|ro|,º|º |u||oº+.
I|e |±||¤±º ¤-¤¤|·¸¤·!-|·|- ¤·-º-¤|c|·¤¤ |º +
W|deºp|e+d |u||+rr+|o|, .eº|cu|o·|u||ouº e|up·
||ou W|e|e e|,||er+|ouº o| e.eu u|||c+||+| º||u
|eº|ouº +|e +ººoc|+|ed W||| |euºe |u||+e |u.o|.|uç
||e ||uu|, ceu||+| |od,, +ud º||u |o|dº |u +dd|||ou
|o ||e e\||er|||eº (||ç. o·20).
C·:c|··:·c| ¤-¤¤|·¸¤·!-|·|- ¤·-º-¤|c|·¤¤ |+º
p|or|ueu| rucoº+| |u.o|.ereu|-e|oº|ouº +ud
ºc+|||uç |u ||e rou||, eºop|+çuº, coujuuc||.+,
+uuº, +ud .+ç|u+.
I|e |¸1 |±||¤±º !-·¤c|¤º·º-|·|- ¤·-º-¤|c|·¤¤
º|oWº .eº|c|eº +||+uçed |u +u +uuu|+| |+º||ou ||+|
+|e |er|u|ºceu| o| ||ue+| |çA |u||ouº de|r+|oº|º,
|n, o| CB|C.
n|º|op+||o|oç, o| |eº|ou+| º||u. ºu|ep|de|r+|
|||º|e|º W||| + c|e+u ºep+|+||ou |e|Weeu ||e ep|·
de|r|º +ud de|r|º.
|rruuop+||o|oç, |e.e+|º ||ue+| |çC (p|uº |çA,
|ç\, |+c|o| B, +ud p|ope|d|u) +| ||e de|r+|·
ep|de|r+| juuc||ou. || º+|| ºp|||·º||u ||| |º pe|·
|o|red, c||cu|+||uç +u|||+ºereu| rer||+ue /oue
+u|||od|eº ||ud ||e ||oo| o| ||e |||º|e|, |u cou||+º|
|o |u||ouº perp||ço|d W|e|e +u|||od|eº +|e
|ouud |o ||e |oo|.
Au|||od|eº |u EBA ºe|+ W||| ||ud |o + 290·||+
|+ud |u weº|e|u ||o|º cou|+|u|uç |,pe \|| co|·
|+çeu. Au E||'A ||+| |º .e|, ºpec|||c |o| +u|||od|eº
|o |,pe \|| co||+çeu |º uoW +.+||+||e.
í·-c|¤-¤| o| EBA |º d||||cu||, p+|||cu|+||, |u p+·
||eu|º W||| ||e c|+ºº|c rec|+uo·|u||ouº p|eºeu·
|+||ou. |+||eu|º +|e |e||+c|o|, |o ||ç| doºeº o|
º,º|er|c ç|ucoco|||co|dº, +/+|||op||ue, re||o||e\·
+|e, +ud c,c|op|oºp|+r|de, W||c| +|e ºoreW|+|
|e|p|u| |u ||e |u||+rr+|o|, B|·|||e |o|r o| ||e
d|ºe+ºe. 'ore EBA p+||eu|º |rp|o.e ou d+pºoue
+ud ||ç| doºeº o| co|c||c|ue. 'uppo|||.e ||e|+p,
|º W+||+u|ed |u +|| p+||eu|º W||| EBA.
|C|·9 . o9+.3
°
|C|·¹0 . |¹2.!
£FI0£kM0I¥SIS 8üII0SA ACÇüISIIA (£8A)
S£CII0N 6 Bu||0u' ||'EA'E' 121
FICük£ 6-20 £pìdermo|ysìs bu||osa acquìsìta I||º |º + ||e |u||ouº perp||ço|d·|||e p|eºeu|+||ou W|||
|euºe |u||+e, e|oº|ouº, +ud c|uº|º ou +u e|,||er+|ouº |+ºe. I|e|e |º e\|euº|.e poº||u||+rr+|o|, p|çreu|+||ou
due |o p|e.|ouº |||º|e||uç.
122
S E C I | 0 N 1
MISC£IIAN£0üS
INFIAMMAI0k¥ 0IS0k0£kS
|||,||+º|º |oºe+ (|k) |º +u +cu|e e\+u||er+|ouº
e|up||ou W||| + d|º||uc||.e ro|p|o|oç, +ud o||eu
W||| + c|+|+c|e||º||c ºe||·||r||ed cou|ºe.
|u|||+||,, + º|uç|e (p||r+|,, o| '|e|+|d¨) p|+que |e·
º|ou de.e|opº, uºu+||, ou ||e ||uu|, ¹ o| 2 Wee|º
|+|e| + çeue|+||/ed ºecoud+|, e|up||ou de.e|opº
|u + |,p|c+| d|º||||u||ou p+||e|u.
I|e eu|||e p|oceºº |er||º ºpou|+ueouº|, |u o
Wee|º.
ke+c||.+||ou o| |ur+u |e|peº.||uº (nn\) 1 +ud
nn\·o |º ||e roº| p|o|+||e c+uºe.
FII¥kIASIS k0S£A (Fk) |C|·9. o9o.+

|C|·¹0. |+2
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset 10-43 yeais, but can occui iaiely
in infants and old peisons.
Seasoo Spiing and fall.
£tìo|o¿y Theie is good evidence that PR is as-
sociated with ieactivation of HHV-7 oi HHV-6,
two closely ielated -heipesviiuses.
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos A single heiald patch pie-
cedes the exanthematous phase; which develops
ovei a peiiod of 1-2 weeks. Piuiitus-absent
(25%), mild (50%), oi seveie (25%).
Skìo Iesìoos Heru|d Putch 80% of patients.
Oval, slightly iaised plaque oi patch 2-5 cm,
salmon-ied, fine collaiette scale at peiipheiy;
may be multiple (Fig. 7-1B).
Erunthem Fine scaling papules and plaques
with maiginal collaiette (Fig. 7-1À). Dull pink
oi tawny. Oval, scatteied, with chaiacteiistic
distiibution with the long axes of the oval
lesions following the lines of cleavage in a
°Chiistmas tiee" pattein (Image 7-1). Lesions
usually confined to tiunk and pioximal aspects
of the aims and legs. Raiely on face.
AtypIcu| PItyrIusIs Rvseu Lesions may be
piesent only on the face and neck. The piimaiy
plaque may be absent, may be the sole manifes-
tation of the disease, oi may be multiple. Most
confusing aie the examples of pityiiasis iosea
with vesicles oi simulating eiythema multi-
foime. This usually iesults fiom iiiitation and
sweating, often as a consequence of inadequate
tieatment ( ¡íìyríasís rosea írríìaìa ).
0IFF£k£NIIAI 0IACN0SIS
Mu|tìp|e Sma|| Sca|ìo¿ F|aques Drug eru¡ìíons
(e.g., captopiil, baibituiates); setonJary sy¡|í|ís
(obtain seiology); guììaìe ¡soríasís (no maiginal
collaiette); sma|| ¡|aque ¡ara¡soríasís ; eryì|ema
mígrans with secondaiy lesions; eryì|ema mu|-
ìí[orme, ìínea tor¡orís .
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Patchy oi diffuse paiakeia-
tosis, absence of gianulai layei, slight acanthosis,
focal spongiosis, micioscopic vesicles. Occa-
sional dyskeiatotic cells with an eosinophilic
homogeneous appeaiance. Edema of deimis,
homogenization of the collagen. Peiivasculai
infiltiate mononucleai cells.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 123
C0ükS£
Spontaneous iemission in 6-12 weeks oi less.
Recuiiences aie uncommon.
MANAC£M£NI
Symptomatìc Oial antihistamines and/oi top-
ical antipiuiitic lotions foi ielief of piuiitus.
Topical glucocoiticoids. May be impioved by
UVB phototheiapy oi natuial sunlight expo-
suie if tieatment is begun in the fiist week of
eiuption. Shoit couise of systemic glucocoi-
ticoids.
FICük£ T-1 Fìtyrìasìs rosea  0.e|.|eW o| e\+u||er o| p||,||+º|º |oºe+ W||| ||e |e|+|d p+|c| º|oWu |u
B. I|e|e +|e p+pu|eº +ud ºr+|| p|+queº W||| o.+| cou||çu|+||ouº ||+| |o||oW ||e ||ueº o| c|e+.+çe. I|e ||ue ºc+||uç
o| ||e º+|rou·|ed p+pu|eº c+uuo| |e ºeeu +| |||º r+çu|||c+||ou, W|||e ||e co||+|e||e o| ||e |e|+|d p+|c| |º qu||e
o|.|ouº  ne|+|d p+|c|. Au e|,||er+|ouº (º+|rou·|ed) p|+que W||| + co||+|e||e ºc+|e ou ||e ||+|||uç edçe o| ||e
+d.+uc|uç |o|de|. Co||+|e||e re+uº ||+| ºc+|e |º +||+c|ed +| pe||p|e|, +ud |ooºe |oW+|d ||e ceu|e| o| ||e |eº|ou.


FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 124
SMAII-FIAÇü£ FAkAFS0kIASIS
(0ICIIAI£ 0£kMAI0SIS), SFF
|C|·9 . o9o.2

|C|·¹0 . |+¹.!
CIINICAI MANIF£SIAII0N
Giadual development ovei months. Raie piuii-
tus. Middle age.
Skìo Iesìoos Round, oval, eiythematous,
yellowish oi fawn-coloied, only minimally
elevated patches, ·5 cm in diametei (Fig. 7-2B).
k+|e e|up||ouº W||| Wo||dW|de occu||euce.
IWo |,peº +|e |ecoçu|/ed. ºr+||·p|+que || +ud
|+|çe·p|+que ||.
|eº|ouº +|e ou|, º||ç|||, |u|||||+|ed, ,e||oW|º| o|
|+Wu·co|o|ed p+|c|eº. |u ºr+||·p|+que || ('||)
|eº|ouº +|e ºr+|| (·5 cr) |ouud |o o.+| o| ||ue+|
roº||, ou ||e ||uu|. |+|çe·p|+que || (|||) +|ºo
o.+| o| |||eçu|+||, º|+ped +ud >5 cr. \+, |e
po||||ode|r+|ouº.
'|| doeº uo| p|oç|eºº |o r,coº|º |uuço|deº
(\|). |||, |, cou||+º|, e\|º|º ou + cou||uuur W|||
p+|c|·º|+çe \| +ud c+u p|oç|eºº |o o.e|| \|.
I|e+|reu| couº|º|º o| |op|c+| ç|ucoco|||co|dº, p|o·
|o||e|+p,, o| p|o|oc|ero||e|+p, (|u\A).
FAkAFS0kIASIS £N FIAÇü£S (FF)
IMAC£ T-1 Fìtyrìasìs rosea: ||º||||u||ou 'C|||º|r+º ||ee¨ p+||e|u ou ||e |+c|
Slight scale and wiinkled suiface with cigaiette-
papei appeaiance. Lineai fingei-like (digitate)
shapes on tiunk, pioximal extiemities, and
buttocks, following lines of cleavage, giving
appeaiance of a hug that left fingeipiints (hence,
Jígíìa| Jermaìosís ) (Fig. 7-2À).
0IFF£k£NIIAI 0IACN0SIS
Pityiiasis iosea, laige-plaque paiapsoiiasis, diug
eiuptions, nummulai eczema, tinea coipoiis,
mycosis fungoides.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 125
FICük£ T-2 0ì¿ìtate dermatosìs (sma||-p|aque parapsorìasìs)  I|e |eº|ouº +|e +º,rp|or+||c, ,e|·
|oW|º| o| |+Wu·co|o|ed, .e|, |||u, We||·de||ued, º||ç|||, ºc+|, p+|c|eº. I|e, +|e o.+| +ud |o||oW ||e ||ueº o| c|e+.·
+çe o| ||e º||u, ç|.|uç ||e +ppe+|+uce o| + '|uç¨ ||+| |e|| ||uçe|p||u|º ou ||e ||uu|. I|e |ouç +\|º o| ||eºe |eº|ouº
o||eu |e+c|eº ro|e ||+u 5 cr.  C|oºe up o| ºr+||e| |eº|ouº º|oW|uç W||u|||uç o| ºu||+ce.


FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 126
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Spongifoim deimatitis
with focal aieas of hypeikeiatosis, paiakeia-
tosis, and exocytosis. In the deimis theie aie
a mild supeificial vasculai lymphohistiocytic
infiltiate (piedominantly CD4- T cells) and
deimal edema.
MANAC£M£NI
No tieatment necessaiy, but patients should
be ieassuied. Disease may be tieated with lu-
biicant oi topical steioids. Bioad-band photo-
theiapy; UVB (311 nm) and PUVA aie highly
effective.
IAkC£-FIAÇü£ FAkAFS0kIASIS (IFF)
|C|·9 . o92.2

|C|·¹0 . |+¹.+
CIINICAI MANIF£SIAII0N
Giadual development ovei months and yeais,
staiting with one oi two plaques. Piuiitus is
iaie; the lesions may disappeai aftei exposuie
to sun in the summei to iecui in the fall and
wintei. Middle age.
Skìo Iesìoos Baiely elevated, eiythematous,
dusky-ied, sometimes yellowish plaques
that aie actually patches (Fig. 7-3 À ), with oi
without slight atiophy and smooth oi slightly
scaling suiface (Fig. 7-3 B ). Ciiculai, >10 cm
in diametei, oi iiiegulai and well defined; and
iandomly scatteied on tiunk, buttocks, bieasts,
oi extiemities.
0IFF£k£NIIAI 0IACN0SIS
Sca|ìo¿ F|aques °Eaily" stages of mycosis fun-
goides. The development of ín[í|ìraìíon in the
lesions, aìro¡|y , and ¡oí|í|oJermaìous t|anges
aie clues to eaily mycosis fungoides.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Nonspecific oi, latei, a
bandlike mononucleai cell infiltiate (CD4-)
with atiophy of the epideimis, vacuolization of
the basal cell layei, capillaiy dilatation. Theie
aie no atypical lymphocytes. Mild exocyto-
sis. Piedominance of CD4- T cells, fiequent
CD7 antigen deficiency, and, in the epideimis,
expiession of class II HLA antigens.
Ferìphera| 8|ood Monoclonal T helpei cells
with skin-homing specificity can be detected.
C0ükS£ AN0 Fk0CN0SIS
The lesions peisist foi life and can piogiess to
mycosis fungoides (see Section 20).
MANAC£M£NI
Iopìca| Tempoiaiy iemission with topical
glucocoiticoids.
Fhototherapy Good iesponses to naiiow-band
311-nm UVB oi PUVA photochemotheiapy.
FICük£ T-3 Iar¿e-p|aque parapsorìasìs (parapsorìasìs eo p|aques)  I|e |eº|ouº +|e +º,rp|or+||c,
We||·de||ued, |ouuded, º||ç|||, ºc+|,, |||u p|+queº o| p+|c|eº. I|e |eº|ouº c+u |e |+|çe| ||+u ¹0 cr +ud +|e ||ç||
|ed·||oWu o| º+|rou·p|u|. I|e|e r+, |e +||op|, |u ºore +|e+º. I|e |eº|ouº |e|e +|e |oc+|ed ou ||e e\||er|||eº
|u| ||e, +|e ro|e corrou|, uo|ed ou ||e ||uu|. I|eºe |eº|ouº ruº| |e c+|e|u||, |o||oWed +ud |epe+|ed ||opº|eº
+|e ueceºº+|, |o de|ec| r,coº|º |uuço|deº. I||º eu|||, r+, |e couº|de|ed +º + p|eº|+çe o| r,coº|º |uuço|deº.
 C|oºe up o| |eº|ouº º|oW|uç r|u|r+| ºc+||uç +ud W||u||ed ºu||+ce.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 12T


FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 128
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset 30-60 yeais.
Sex Females > males.
kace Hypeitiophic LP moie common in
blacks.
£tìo|o¿y Idiopathic in most cases but cell-
mediated immunity plays a majoi iole. Majoi-
ity of lymphocytes in the infiltiate aie CD8-
and CD45Ro- (memoiy) cells. Diugs, metals
(gold, meicuiy), oi infection ¦hepatitis C viius
(HCV)] iesult in alteiation in cell-mediated im-
munity. Theie could be HLA-associated genetic
susceptibility that would explain a piedisposi-
tion in ceitain peisons. Lichenoid lesions of
chionic giaft-veisus-host disease (GVHD) of
skin aie indistinguishable fiom those of LP (see
Section 21).
CIINICAI MANIF£SIAII0N
0oset Acute (days) oi insidious (ovei weeks).
Lesions last months to yeais, asymptomatic oi
piuiitic; sometimes seveie piuiitus. Mucous
membiane lesions aie painful, especially when
ulceiated.
Skìo Iesìoos Papules, flat-topped, 1 to 10 mm,
shaiply defined, shiny (Fig. 7-4). Violaceous, with
white lines (Wickham stiiae) (Fig. 7-4 À ), seen
best with hand lens aftei application of mineial
oil. Polygonal oi oval (Fig. 7-4 B ). Giouped
(Figs. 7-4 and 7-5), annulai, oi disseminated
scatteied disciete lesions when geneialized
(Fig. 7-6). In daik-skinned individuals,
postinflammatoiy hypeipigmentation is
common. May piesent on lips (Fig. 7-7 À ) and
in a lineai aiiangement aftei tiauma (Koebnei
wo||dW|de occu||euce, |uc|deuce |eºº ||+u oue
pe|ceu|, +|| |+ceº.
|| |º +u +cu|e o| c||ou|c |u||+rr+|o|, de|r+|oº|º
|u.o|.|uç º||u +ud/o| rucouº rer||+ueº.
C|+|+c|e||/ed |, ||+|·|opped (|+||u ¤|c¤±º , '||+|¨),
p|u| |o .|o|+ceouº, º||u,, p|u||||c po|,çou+| p+·
pu|eº. I|e |e+|u|eº o| ||e |eº|ouº |+.e |eeu deº·
|çu+|ed +º ||e |ou| |'º-p+pu|e, pu|p|e, po|,çou+|,
p|u||||c.
||º||||u||ou. p|ed||ec||ou |o| ||e\u|+| +ºpec|º o|
+|rº +ud |eçº, c+u |ecore çeue|+||/ed.
|u ||e rou|| r|||,·W|||e |e||cu|+|ed p+pu|eº, r+,
|ecore e|oº|.e +ud e.eu u|ce|+|e.
\+|u º,rp|or. p|u|||uº, |u ||e rou||, p+|u.
I|e|+p,. |op|c+| +ud º,º|er|c ç|ucoco|||co|dº,
c,c|oºpo||ue.
IICh£N FIANüS (IF) |C|·9 . o91.0

|C|·¹0 . |+!
oi isomoiphic phenomenon (Fig. 7-7 B ).
SItes v] PredI|ectIvn Wiists ¦flexoi (Fig.
7-4À )], lumbai iegion, shins ¦thickei, hypei-
keiatotic lesions (Fig. 7-5 B )], scalp, glans penis
(See Figs. 35-9, 35-10), mouth (Image 7-2).
varìaots
HypertrvphIc Laige thick plaques aiise on the
foot, doisum of hands (Fig. 7-5 À ), and shins
(Fig. 7-5 B ); moie common in black males.
Although typical LP papule is smooth, hypei-
tiophic lesions may become hypeikeiatotic.
AtrvphIc White-bluish, well-demaicated pa-
pules and plaques with cential atiophy.
Fv||Icu|ur Individual keiatotic-folliculai pa-
pules and plaques that lead to cicatiicial alo-
pecia. Spinous folliculai lesions, typical skin and
mucous membiane LP, and cicatiicial alopecia
of the scalp (See Figs. 32-19, 32-20) aie called
Cra|am Líìì|e synJrome . (See Section 32.)
VesIcu|ur Vesiculai oi bullous lesions may
develop within LP patches oi independent of
them within noimal-appeaiing skin. Theie aie
diiect immunofluoiescence findings consist-
ent with bullous pemphigoid, and the seia of
these patients contain bullous pemphigoid IgG
autoantibodies (see Section 6).
PIgmentvsus Hypeipigmented, daik-biown
macules in sun-exposed aieas and flexuial folds.
In Latin Ameiicans and othei daik-skinned
populations. Significant similaiity with ashy
deimatosis (see Section 13).
ActInIcus Papulai LP lesions aiise in sun-
exposed sites, especially the doisa of hands and
aims.
U|cerutIve LP may lead to theiapy-iesistant
ulceis, paiticulaily on the soles, iequiiing skin
giafting.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 129

FICük£ T-4 Iìcheo p|aous  ||+|·|opped, po|,çou+|, º|+|p|, de||ued p+pu|eº o| .|o|+ceouº co|o|, ç|ouped
+ud cou||ueu|. 'u||+ce |º º||u, +ud, upou c|oºe |uºpec||ou W||| + |+ud |euº, ||ue W|||e ||ueº +|e |e.e+|ed (w|c|·
|+r º|||+e, +||oW).  C|oºe up o| ||+|·|opped º||u, .|o|+ceouº p+pu|eº ||+| +|e po|,çou+|.

FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 130
Mucous Membraoes Some 40-60% of individ-
uals with LP have oiophaiyngeal involvement
(see Section 34).
RetIcu|ur LP Reticulate (netlike) pattein of
lacy white hypeikeiatosis on buccal mucosa (see
Fig. 34-3), lips (Fig. 7-7À), tongue, gingiva; the
most common pattein of oial LP.
ErvsIve vr U|cerutIve LP Supeificial eiosion
with/without oveilying fibiin clot; occuis on
tongue and buccal mucosa (see Fig. 34-3); shiny
ied painful eiosion of gingiva (desquamative
gingivitis) (see Fig. 34-5) oi lips (Fig. 7-7À).
Caicinoma may veiy iaiely develop in mouth
lesions.
Ceoìta|ìa Papulai (see Figs. 35-9, 35-10) agmi-
nated, annulai, oi eiosive lesions aiise on penis
(especially glans), sciotum, labia majoia, labia
minoia, vagina.
haìr aod Naì|s Scu|p Folliculai LP, atiophic
scalp skin with scaiiing alopecia (See Figs. 32-
19, 32-20). (See Section 32.)
NuI|s Destiuction of nail fold and nail bed
with longitudinal splinteiing (see Fig. 33-10).
IICh£N FIANüS-IIk£ £küFII0NS
Lichen planus-like eiuptions closely mimic
typical LP, both clinically and histologically.
They occui as a clinical manifestation of chionic
GVHD, in deimatomyositis, and as cutaneous
manifestations of malignant lymphoma but
may also develop as the iesult of theiapy with
ceitain diugs and aftei industiial use of ceitain
compounds (Table 7-1).
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical findings confiimed by histopathology.
Skìo Iesìoos Pupu|ur LP Chionic cutane-
ous lupus eiythematosus, psoiiasis, pityiia-
sis iosea, eczematous deimatitis, lichenoid
GVHD; single lesions: supeificial basal cell
caicinoma, Bowen disease (in situ squamous
cell caicinoma).
HypertrvphIc LP Psoiiasis vulgaiis, lichen
simplex chionicus, piuiigo nodulaiis, stasis
deimatitis, Kaposi saicoma.
Drug-1nduced LP See Table 7-1.
Mucous Membraoes Leukoplakia, pseu-
domembianous candidiasis (thiush), HIV-asso-
ciated haiiy leukoplakia, lupus eiythematosus,
bite tiauma, mucous patches of secondaiy
syphilis, pemphigus vulgaiis, bullous pemphi-
goid (see Section 34).
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Inflammation with hypei-
keiatosis, incieased gianulai layei, iiiegulai
acanthosis, liquefaction degeneiation of the
basal cell layei, and bandlike mononucleai in-
filtiate that hugs the epideimis. Keiatinocyte
apoptosis (colloid, Civatte bodies) is found at
the deimal-epideimal junction. Diiect immun-
ofluoiescence ieveals heavy deposits of fibiin at
the junction and IgM and, less fiequently, IgA,
IgG, and C3 in the colloid bodies.
C0ükS£
Cutaneous LP usually peisists foi months, but
in some cases, foi yeais; hypeitiophic LP on the
shins and oial LP often foi decades. The inci-
dence of oial squamous cell caicinoma in indi-
viduals with oial LP is incieased (5%); patients
should be followed at iegulai inteivals.
MANAC£M£NI
Ioca| Iherapy
G|ucvcvrtIcvIds Topical glucocoiticoids with
occlusion foi cutaneous lesions. Intialesional
tiiamcinolone (3 mg/mL) is helpful foi symp-
tomatic cutaneous oi oial mucosal lesions and
lips.
Cyc|vspvrIne und Tucrv|Imus Sv|utIvns Re-
tention °mouthwash" foi seveiely symptomatic
oial LP.
Systemìc Iherapy
Cyc|vspvrIne In veiy iesistant and geneialized
cases, 5 mg/kg pei day will induce iapid iemis-
sion, quite often not followed by iecuiience.
G|ucvcvrtIcvIds Oial piednisone is effective
foi individuals with symptomatic piuiitus,
painful eiosions, dysphagia, oi cosmetic disfig-
uiement. A shoit, tapeied couise is piefeiied:
70 mg initially, tapeied by 5 mg/day.
SystemIc RetInvIds (AcItretIn) 1 mg/kg pei
day is helpful as adjunctive measuie in seveie
(oial, hypeitiophic) cases, but usually addi-
tional topical tieatment is iequiied.
FüvA Fhotochemotherapy
In individuals with geneialized LP oi cases
iesistant to topical theiapy.
0ther Ireatmeots
Mycophenolate mofetil, hepaiin analogues
(enoxapaiin) in low doses have antipiolifeiative
and immunomodulatoiy piopeities; azathio-
piine.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 131
FICük£ T-5 hypertrophìc |ìcheo p|aous  Cou||ueu| |,pe||e|+|o||c p+pu|eº +ud p|+queº ou ||e do|ºur
o| ||e |+ud o| + ||ç||·co|o|ed r+u o| A|||c+u deºceu|. n,pe||e|+|oº|º co.e|º w|c||+r º|||+e, +ud ||e c|+|+c|e||º||c
.|o|+ceouº co|o| o| ||e |eº|ouº c+u |e ºeeu ou|, +| ||e .e|, r+|ç|uº.  n,pe|||op||c ||c|eu p|+uuº ou ||e |oWe|
|eç o| + 50·,e+|·o|d r+u o| A|+||+u deºceu|. |eº|ouº |o|r |||c| p|+queº o| + d+|| ||oWu .|o|+ceouº co|o| +ud
|+.e + |,pe||e|+|o||c ºu||+ce.
IA8I£ T-1 A¿ents |nducin¿ Lichen P|anus and Lichenoid Reactions
Corrou |uduce|º
Co|d º+||º
||oc|e|º
Au||r+|+||+|º
I||+/|de d|u|e||cº
|u|oºer|de
'p||ouo|+c|oue
|eu|c|||+r|ue
|eºº corrou
ACE |u|||||o|º
C+|c|ur c|+uue| ||oc|e|º
'u||ou,|u|e+
|ouº|e|o|d+| +u||·|u||+rr+|o|,
d|uçº
Ke|ocou+/o|e
Ie||+c,c||ue
||euo|||+/|ue
'u||+º+|+/|ue
C+||+r+/ep|ue
|||||ur
|eºº corrou
Au|||u|e|cu|oº|º d|uçº
|od|deº
k+d|ocou||+º| red|+
\e||,|dop+
ne+., re|+|º
|uduce|º o| ||c|eu p|+uuº |,
cou|+c|
Co|o| |||r de.e|ope|º
|eu|+| |eº|o|+||.e r+|e||+|º
\uº| +r||e||e
||c||e
Co|d
|uduce|º o| p|o|od|º||||u|ed
||c|euo|d e|up||ou
5·||uo|ou|+c||
C+||+r+/ep|ue
C||o|p|or+/|ue
||+/o\|de
E||+r|u|o|
|uduce|º o| p|o|od|º||||u|ed
||c|euo|d e|up||ou (:¤¤|·¤±-!)
|,||||uo|
0u|u|ue
0u|u|d|ue
Ie||+c,c||ue
I||+/|de
|u|oºer|de
|uduce|º o| o|+| ||c|eu p|+uuº
+ud ||c|euo|d e|up||ou
A||opu||uo|
ACE |u|||||o|º
C,+u+r|de
|eu|+| |eº|o|+||.e r+|e||+|º
Co|d º+||º
Ke|ocou+/o|e
|ouº|e|o|d+| +u||·|u||+rr+|o|,
d|uçº
|eu|c|||+r|ueº
'u||ou,|u|e+
ACE, +uç|o|euº|u·cou.e|||uç eu/,re.
 
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 132
FICük£ T-6 0ìssemìoated |ìcheo p|aous A º|oWe| o| d|ººer|u+|ed p+pu|eº ou ||e ||uu| +ud ||e e\||er|·
||eº (uo| º|oWu) |u + +5·,e+|·o|d ||||p|uo. |ue |o ||e e||u|c co|o| o| ||e º||u, ||e p+pu|eº +|e uo| +º .|o|+ceouº +º
|u C+uc+º|+uº |u| |+.e + ||oWu|º| |ue.
IMAC£ T-2 Iìcheo p|aous:
p|ed||ec||ou º||eº.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 133


FICük£ T-T Iìcheo p|aous  '||.e|,·W|||e, cou||ueu|, ||+|·|opped p+pu|eº ou ||e ||pº. |o|e. w|c||+r
º|||+e (+||oW). B. ||c|eu p|+uuº, Koe|ue| p|euoreuou. ||ue+| +||+uçereu| o| ||+|·|opped, º||u, p+pu|eº ||+|
e|up|ed +||e| ºc|+|c||uç.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 134
£FI0£MI0I0C¥
Common.
A¿e oI 0oset Childien and young adults.
Sex Female:male iatio 2:1.
£II0I0C¥ AN0 FAIh0C£N£SIS
Unknown. An immunologically mediated
neciotizing inflammation that suiiounds blood
vessels, alteiing collagen and elastic tissue. Gen-
eialized GA may be associated with diabetes
mellitus.
CIINICAI MANIF£SIAII0N
Duiation months to yeais. Usually asympto-
matic and only cosmetic disfiguiement.
Skìo Iesìoos Fiim, smooth, shiny, beaded,
deimal papules and plaques, 1-5 cm annulai,
aicifoim plaques with cential depiession
(see Fig. 7-8À, B), skin-coloied, violaceous,
eiythematous. Su|tuìaneous CÀ (iaie): painless,
skin-coloied, deep deimal oi subcutaneous,
solitaiy oi multiple nodules.
DIstrIhutIvn Isolated lesion, paiticulaily
on doisum of hand, fingei, oi lowei aim
(Fig. 7-8 À ), multiple lesions on extiemities
and tiunk (Fig. 7-8 B ), oi geneialized (papulai;
oldei patients) (Fig. 7-8 C ). Subcutaneous le-
sions aie located neai joints, palms and soles,
buttocks.
varìaots
· Per[oraìíng lesions aie veiy iaie and mostly on
the hands; cential umbilication followed by
ciusting and ulceiation; this type was associ-
ated with diabetes in one seiies.
· May iaiely involve fascia and tendons, causing
scleiosis.
· Geneialized GA: in this foim a seaich foi
diabetes mellitus should be made.
A corrou ºe||·||r||ed, +º,rp|or+||c, c||ou|c
de|r+|oº|º o| ||e de|r|º.
uºu+||, occu|º |u c|||d|eu +ud ,ouuç +du||º.
Couº|º|º o| p+pu|eº |u +u +uuu|+| +||+uçereu|,
corrou|, +||º|uç ou ||e do|º+ o| ||e |+udº +ud
|ee|, e||oWº, +ud |ueeº.
'ore||reº |ecoreº çeue|+||/ed |u d|º||||u||ou.
uu|eºº d|º||çu||uç, uo ||e+|reu| |º +u op||ou.
CkANüI0MA ANNüIAk£ (CA) |C|·9 . o95.39

|C|·¹0 . |92.0
0IFF£k£NIIAI 0IACN0SIS
GA is impoitant because of its similaiity to
moie seiious conditions.
Fapu|ar Iesìoos aod F|aques Neciobiosis
lipoidica, papulai saicoid, lichen planus, lym-
phocytic infiltiate of Jessnei.
Subcutaoeous Nodu|es Rheumatoid nodules:
confusion can occui because of the similai
pathology of GA and iheumatic nodule oi
iheumatoid nodules. Also subcutaneous fungal
infections such as spoiotiichosis and NTM (M.
marínum).
Aoou|ar Iesìoos Tinea, eiythema migians,
saicoid, lichen planus.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Foci of chionic inflamma-
toiy and histiocytic infiltiations in supeificial
and mid-deimis, with neciobiosis of connective
tissue suiiounded by a wall of palisading histio-
cytes and multinucleated giant cells.
C0ükS£
The disease disappeais in 75% of patients in
2 yeais. Recuiiences aie common (40%), but
they also disappeai.
MANAC£M£NI
GA is a local skin disoidei and not a maikei foi
inteinal disease, and spontaneous iemission is
the iule. No ìreaìmenì ís an o¡ìíon í[ ì|e |esíons are
noì Jís[íguríng . Lesions may iesolve aftei biopsy.
Iopìca| Iherapy TvpIcu| G|ucvcvrtIcvIds -
Applied undei plastic occlusion oi hydiocolloid.
1ntru|esIvnu| TrIumcInv|vne 3 mg/mL into
lesions is veiy effective.
Cryvspruy Supeificial lesions iespond to liq-
uid nitiogen, but atiophy may occui.
FüvA Fhotochemotherapy Effective in genei-
alized GA.
Systemìc C|ucocortìcoìds Effective in geneial-
ized GA, but iecuiiences common.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 135


FICük£ T-8 Craou|oma aoou|are  Cou||ueu|, pe+||,·W|||e p+pu|eº |o|r|uç + We||·der+|c+|ed ||uç W|||
ceu||+| |eç|eºº|ou.  \u|||p|e ç|+uu|or+|+ |o|r|uç +uuu|+| +ud ºer|c||cu|+| p|+queº W||| ceu||+| |eç|eºº|ou ou
||e +|r o| + +5·,e+|·o|d r+u o| A|||c+u e\||+c||ou.  ||ººer|u+|ed ç|+uu|or+ +uuu|+|e |u + C+uc+º|+u. \u|||p|e,
We||·de||ued, pe+||,·W|||e p+pu|eº, ºore o| W||c| º|oW + ceu||+| dep|eºº|ou

FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 136
£FI0£MI0I0C¥ AN0 £II0I0C¥
Iocìdeoce Raie between the ages of 20 and 50;
in lineai moiphea, eailiei. Panscleiotic Moi-
phea, a disabling disoidei, usually staits befoie
age 14.
Sex Females aie affected about thiee times as
often as males, including childien. Lineai scle-
iodeima is the same in males and females.
£tìo|o¿y Unknown. At least some patients
(piedominantly in Euiope) with classic moi-
phea have scleiosis due to Borre|ía |urgJor[erí
infection, and, if not too scleiotic, the lesions
can disappeai with piolonged couises of oial
antibiotics. Pigmentation, howevei, peisists.
Moiphea has been noted aftei x-iiiadiation
foi bieast cancei. Moiphea is not ielated to
systemic scleiodeima.
CIASSIFICAII0N 0F vAkI0üS I¥F£S 0F
I0CAIII£0 SCI£k00£kMA
· Círtumstrí|eJ . plaques oi bands
· Matu|ar. small, confluent patches
· Línear st|eroJerma . uppei oi lowei extiemity
· Fronìo¡aríeìa| (en tou¡ Je sa|re)
· Cenera|í:eJ mor¡|ea
· Panst|eroìít . involvement of deimis, fat, fascia,
muscle, bone.
CIINICAI MANIF£SIAII0N
Symptoms Usually none. No histoiy of
Raynaud phenomenon. Lineai and panscleiotic
moiphea can iesult in majoi facial oi limb
asymmetiy, flexion contiactuies, and disability.
Can cause seveie disfiguiement.
Skì o Fì odì o¿s P|aques -ciicumsciibed,
induiated, haid, but pooily defined aieas of
skin; 2-15 cm in diametei, iound oi oval, often
bettei felt than seen. Initially, puiplish oi mauve.
A |oc+||/ed +ud c||curºc|||ed cu|+ueouº ºc|e|oº|º
c|+|+c|e||/ed |, e+||, .|o|+ceouº, |+|e| |.o|,·
co|o|ed, |+|deued º||u.
\+, |e ºo|||+|,, ||ue+|, çeue|+||/ed, +ud, |+|e|,, +c·
corp+u|ed |, +||op|, o| uude||,|uç º||uc|u|eº.
|| |º uu|e|+|ed |o º,º|er|c ºc|e|ode|r+.
',¤¤¤,¤º |oc+||/ed ºc|e|ode|r+, c||curºc|||ed
ºc|e|ode|r+.
M0kFh£A |C|·9 . 10¹.0

|C|·¹0 . |9+.0
In time, suiface becomes smooth and shiny
aftei months to yeais, ivoiy with lilac-coloied
edge °lilac iing" (Fig. 7-9). May have hypei- and
hypopigmentation in involved scleiotic aieas
(Fig. 7-10). Raiely, lesions become atiophic
and hypeipigmented without going thiough
a scleiotic stage (atiophodeima of Pasini and
Pieiini) (see Fig. 7-13B).
DIstrIhutIvn
Círtumstrí|eJ. Tiunk (Fig. 7-9), limbs, face,
genitalia; less commonly, axillae, peii-
neum, aieolae.
Cenera|í:eJ . Initially on tiunk (uppei, bieasts,
abdomen) (Fig. 7-10) thighs.
Línear . Usually on extiemity (Fig. 7-11) oi
[ronìo¡aríeìa|-scalp and face (Fig. 7-12);
heie it may iesemble a scai fiom a stiike
with a sabei ( en tou¡ Je sa|re ).
Matu|ar. Small (<3 mm) maculai patches,
confluent (Fig. 7-13 À ); clinically indistin-
guishable fiom lichen scleiosus et atiophi-
cus (see p. 142).
Àìro¡|ít. Atiophodeima of Pasini and Pieiini
(Fig. 7-13 B ).
Panst|eroìít . On tiunk (Fig. 7-14) oi extiemi-
ties.
Mouth With lineai moiphea of head, may
have associated hemiatiophy of tongue.
haìr aod Naì|s Scaiiing alopecia with scalp
plaque. Paiticulaily with lineai moiphea of the
head. Nail dystiophy in lineai lesions of extiem-
ity oi in panscleiotic moiphea.
Ceoera| £xamìoatìoo
Moiphea aiound joints and lineai moiphea
may lead to flexion contiactuies. Panscleiotic
moiphea is associated with atiophy and fibiosis
of muscle (Fig. 7-14). Extensive involvement of
tiunk may iesult in iestiicted iespiiation. With
lineai moiphea of the head (Fig. 7-12), theie
may be associated atiophy of oculai stiuctuies
and atiophy of bone. Noìe. moiphea may be as-
sociated with lichen scleiosus et atiophicus.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 13T
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical, confiimed by biopsy. Scleiotic plaque
associated with B. |urgJor[erí infection, acio-
deimatitis chionica atiophicans, piogiessive
systemic scleiosis, lichen scleiosus et atiophi-
cus, eosinophilic fasciitis, toxic oil syndiome,
eosinophilia-myalgia syndiome associated with
L-tiyptophan ingestion, scleiedema, Paiiy-
Rombeig syndiome (hemiatiophy).
FICük£ T-9 Morphea I||º |º +u |udu|+|ed |.o|,·co|o|ed, º||u, p|+que W||| + |||+c·co|o|ed, |||·de||ued |o|de|
(+||oWº). \oº| |eº|ouº +|e |e||e| |e|| ||+u ºeeu |ec+uºe ||e, +|e |udu|+|ed.
IA80kAI0k¥ £XAMINAII0NS
Sero|o¿y Appiopiiate seiologic testing to iule
out B. |urgJor[erí infection.
0ermatopatho|o¿y Epideimis appeais noi-
mal to atiophic with loss of iete iidges. Deimis
edematous with homogeneous and eosinophilic
collagen. Slight infiltiate, peiivasculai oi dif-
fuse; lymphocytes, plasma cells, maciophages.
Latei, deimis thickened with few fibioblasts
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 138
and dense collagen; inflammatoiy infiltiate at
deimal-subcutis junction; deimal appendages
disappeai piogiessively. Panscleiotic lesions
show fibiosis and disappeaiance of subcutane-
ous tissue, with fibiosis involving fascia. Silvei
stains should be peifoimed to iule out B. |urg-
Jor[erí infection. Histopathology distinct fiom
that of lichen scleiosus et atiophicus.
0IACN0SIS
Clinical diagnosis, usually confiimed by skin
biopsy.
C0ükS£
May be slowly piogiessive; °buin out" and
spontaneous iemissions can iaiely occui.
FICük£ T-10 Morphea |||eçu|+|, ||oWu|º|, |udu|+|ed |eº|ouº W||| |oc+| |.o|,·co|o|ed r+cu|+| |eº|ouº ou ||e
|e|| ||p. '|r||+| |eº|ouº We|e +|ºo |ouud ou ||e c|eº| +ud ou ||e |+c|.
MANAC£M£NI
Theie is no effective tieatment foi moiphea, but
some iepoits of tieatment aie as follows:
Morphea-Iìke Iesìoos Assocìated wìth Iyme
8orre|ìosìs In patients with eaily involvement,
theie may be a ieveisal of scleiosis with high-
dose paienteial penicillin oi ceftiiaxone; tieat-
ment given in seveial couises ovei a time span
of seveial months. Best iesponse if combined
with oial glucocoiticoids.
Fhototherapy wìth üvA-1 (340-400 om) In
oui expeiience, the tieatment is not easy oi veiy
successful because of the piolonged iiiadiation
times and the disfiguiing hypeipigmentation of
the iiiadiated aieas.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 139
FICük£ T-11 Iìoear Morphea
|udu|+|ed, |.o|,·W|||e |eº|ou e\|eud|uç
||or uppe| |||ç| |o ||e do|ºur o| ||e |oo|.
|udu|+||ou |º p|ououuced, +ud |u ||e |eç|ou
+|o.e ||e |uee || e\|eudº |o ||e |+ºc|+ (p+u·
ºc|e|o||c ro|p|e+). || p|oç|eºº|.e, || W||| ||r||
||e ro.ereu| o| ||e jo|u|.
FICük£ T-12 Iìoear morphea,
"eo coup de sabre" IWo ||u·
e+|, p+|||+||, |.o|,·W|||e (ou ||e
ºc+|p) +ud |,pe|p|çreu|ed (ou
||e |o|e|e+d) dep|eººed |eº|ouº
e\|eud|uç ||or ||e c|oWu o| ||e
|e+d, W|e|e ||e, |+.e |ed |o
+|opec|+, o.e| ||e |o|e|e+d |o ||e
o||||+. I|e, |oo| |||e ºc+|º +||e|
º||||eº W||| + º+|e|, |euce ||e
||euc| deº|çu+||ou. I|eºe |eº|ouº
c+u e\|eud |o ||e |oue +ud |+|e|,
|o ||e du|+ r+|e|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 140

FICük£ T-13 Macu|ar Iorm oI morphea  I|e|e +|e ru|||p|e, º||u|uç, |.o|,·W|||e r+cu|eº W||| cou||u·
euce |e+d|uç |o + |e||cu|+|ed p+||e|u. I|eºe |eº|ouº +|e |+||e| ºupe|||c|+| +ud ||e|e|o|e |eºº |udu|+|ed. Au |rpo|·
|+u| d|||e|eu||+| d|+çuoº|º |º ||c|eu ºc|e|oºuº e| +||op||cuº.  A||op||c, |,pe|p|çreu|ed |o|r o| ro|p|e+ (c+||ed
+||op|ode|r+ o| |+º|u| +ud ||e||u|). I|e|e |º + d|||uºe ||oWu +ud º|+|p|, de||ued |,pe|p|çreu|+||ou W||| + |eºº
p|çreu|ed |o|||cu|+| p+||e|u. I|eºe |eº|ouº +|e +||op||c +ud uo| |udu|+|ed.

S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 141
FICük£ T-14 Faosc|erotìc morphea I||º |,pe +||ec|º +|| |+,e|º o| ||e º||u |uc|ud|uç ||e |+ºc|+ +ud e.eu
ruºc|e. I|e º||u |º ç||º|eu|uç, |,pe|p|çreu|ed, +ud |+|d +º Wood. || |º o|.|ouº ||+| p+uºc|e|o||c ro|p|e+ |e+dº
|o couº|de|+||e |uuc||ou+| |rp+||reu|. || ||eºe |eº|ouº occu| ou ||e uppe| ||uu|, ||e, c+u |rp+|| e\cu|º|ou o| ||e
c|eº| +ud ||uº ||e+|||uç.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 142
|'A |º + c||ou|c +||op||c d|ºo|de| r+|u|, o| ||e
+uoçeu||+| º||u o| |er+|eº |u| +|ºo o| r+|eº +ud
o| ||e çeue|+| º||u.
A d|ºe+ºe o| +du||º, |u| +|ºo occu|||uç |u c|||d|eu
¹-¹! ,e+|º o| +çe. |er+|eº |eu ||reº ro|e o||eu
+||ec|ed ||+u r+|eº.
w||||º|, |.o|, o| po|ce|+|u·W|||e, º|+|p|, der+|·
c+|ed, |ud|.|du+| p+pu|eº r+, |ecore cou||ueu|,
|o|r|uç ¤|c¡±-º (||ç. 1·¹5). 'u||+ce o| |eº|ouº
r+, |e e|e.+|ed o| |u ||e º+re p|+ue +º uo|r+|
º||u, o|de| |eº|ouº r+, |e dep|eººed. |||+|ed
p||oºe|+ceouº o| ºWe+| duc| o||||ceº ||||ed W|||
|e|+||u p|uçº (de||º), || p|uçç|uç |º r+||ed, ºu||+ce
+ppe+|º |,pe||e|+|o||c (||ç. 1·¹5C).
3±||c- +ud -·¤º·¤¤º occu| +ud ¤±·¤±·c |º o||eu +
c|+|+c|e||º||c +ud |deu|||,|uç |e+|u|e (||ç. 1·¹5B),
|-|c¤¸·-:|cº·c.
|eº|ouº occu| ou çeue|+| º||u o| ou ||e çeu||+||+.
0u .u|.+, |,pe||e|+|o||c p|+queº r+, |ecore
e|oº|.e, r+ce|+|ed, .u|.+ r+, |ecore +||op||c,
º||uu|eu, eºpec|+||, c|||o||º +ud |+||+ r|uo|+, W|||
.+ç|u+| |u||o||uº |educed |u º|/e (ºee ||ç. !5·¹o).
|uº|ou o| |+||+ r|uo|+ +ud r+jo|+.
|u uuc||curc|ºed r+|eº, p|epuce |||º| º|oWº
|.o|, W|||e cou||ueu| p+pu|eº (ºee ||çº. !5·¹2,
!5·¹+) |u| ||eu |ecoreº ºc|e|o||c +ud c+uuo| |e
|e||+c|ed ( ¤|·¤¤º·º ). C|+uº +ppe+|º |.o|, o| po|·
ce|+|u·W|||e, ºer|||+uºp+|eu|, |eºer|||uç ro||e|·
o|·pe+|| W||| +dr|\ed pu|pu||c |ero|||+çeº.
|ouçeu||+| |'A uºu+||, +º,rp|or+||c, çeu||+|
º,rp|or+||c. |u |er+|eº, .u|.+| |eº|ouº r+, |e
ºeuº|||.e, eºpec|+||, W|||e W+|||uç, p|u|||uº, p+|u·
|u|, eºpec|+||, || e|oº|ouº +|e p|eºeu|, d,ºu||+, d,º·
p+|euu|+. |u r+|eº, |ecu||eu| |+|+u|||º, +cqu||ed
p||roº|º.
I|e ||º|op+||o|oç, |º d|+çuoº||c W||| + deuºe
|,rp|oc,||c |u|||||+|e |uçç|uç ||e |u|||+||, |,pe|·
||op||c +ud |+|e|, +||op||c ep|de|r|º +ud ||eu
º|u||uç doWu |u|o ||e de|r|º, |e|uç ºep+|+|ed
||or ||e ep|de|r|º |, +u eder+|ouº, º||uc|u|e·
|eºº ºu|ep|de|r+| /oue.
I|e e||o|oç, o| |'A |º uu|uoWu, |u| |epo||º ||or
Eu|ope |+.e docureu|ed +u +ººoc|+||ou o| ||A
o| 3¤··-|·c ºpp. W||| |'A |u c+ºeº ||or Ce|r+u,
+ud l+p+u, ||A o| ||e ºp||oc|e|eº de|ec|ed |u
||eºe p+||eu|º W+º uo| |ouud |u +u, o| ||e Are||·
c+u º+rp|eº.
I|e cou|ºe o| |'A W+\eº +ud W+ueº. |u ç|||º ||
r+, uude|ço ºpou|+ueouº |eºo|u||ou, |u Woreu
|| |e+dº |o +||op|, o| ||e .u|.+ +ud |u reu |o
p||roº|º. |+||eu|º º|ou|d |e c|ec|ed |o| ||e oc·
cu||euce o| ºqu+rouº ce|| c+|c|uor+ o| ||e .u|.+
+ud peu|º.
\+u+çereu| |º .e|, |rpo||+u|, +º |||º d|ºe+ºe c+u
c+uºe + de.+º|+||uç +||op|, o| ||e |+||+ r|uo|+
+ud c|||o|+| |ood. |o|eu| |op|c+| ¸|±:¤:¤·|·:¤·!
¤·-¤c·c|·¤¤º (c|o|e|+ºo| p|op|ou+|e) |+.e p|o.ed
e||ec||.e |o| çeu||+| |'A +ud º|ou|d |e uºed |o|
o-3 Wee|º ou|,. |+||eu|º º|ou|d |e rou||o|ed
|o| º|çuº o| ç|ucoco|||co|d·|uduced +||op|,. |·¤-·
:·¤|·¤±º +ud |c:·¤|·¤±º +|e +|roº| +º e||ec||.e.
í¤¤·:c| c¤!·¤¸-¤º +|e |eºº uºed uoW |ec+uºe
||e, c+u ºore||reº c+uºe + c|||o|+| |,pe|||op|,.
',º|-¤·: ||-·c¤, . |,d|oc||o|oqu|ue, ¹25-¹50
rç/d, |o| Wee|º |o + |eW rou||º (rou||o| |o|
ocu|+| º|de e||ec|º).
|u r+|eº, :··:±¤:·º·¤¤ |e||e.eº º,rp|orº o| p||·
roº|º +ud |u ºore c+ºeº c+u |eºu|| |u |er|ºº|ou.
IICh£N SCI£k0SüS et AIk0FhICüS (ISA)
|C|·9 . 10¹.0

|C|·¹0 . |90.0
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 143


FICük£ T-15 Iìcheo sc|ero-
sus et atrophìcus  \u|||p|e,
|.o|,·W|||e, |udu|+|ed +ud º||ç|||,
|,pe|·|e|+|o||c p+pu|eº ou ||e c|eº|
o| + +2·,e+|·o|d Wor+u.  I|e |.o|,·
W|||e p+pu|eº o| ||c|eu ºc|e|oºuº |e|e
|+.e re|çed |o |o|r + ºupe|||c|+||,
|udu|+|ed +ud +|ºo +||op||c p|+que
o| º|+|p r+|ç|u+||ou. nero|||+çe |u
||e ceu|e| o| |||º p|+que |º +u |rpo|·
|+u| d|||e|eu||+| d|+çuoº||c º|çu |o ||e
r+cu|+| |o|r o| ro|p|e+  ||c|eu
ºc|e|oºuº |u ||e ç|o|u |eç|ou o| +
o0·,e+|·o|d |er+|e. ne|e ||e p+pu|eº
|+.e re|çed |o |o|r + |+|çe |,pe|·
|e|+|o||c p|+que W||| º|+|p de||u|||ou.
I|e|e +|e +|ºo c|uº|º |eºu|||uç ||or
e|oº|ouº.

FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 144
||| +|e d|º||uçu|º|ed |, ||e|| c||u|c+| c|+|+c|e||º·
||cº, |+.|uç |deu||c+| de|r+|op+||o|oç|c ||ud|uçº,
+ud |uc|ude.
':|+r|e|ç d|ºe+ºe , +|ºo |uoWu +º p|oç|eºº|.e
p|çreu|ed pu|pu||c de|r+|oº|º o| p|oç|eºº|.e
p|çreu|+|, pu|pu|+
\+jocc|| d|ºe+ºe , +|ºo |uoWu +º pu|pu|+ +u·
uu|+||º |e|+uç|ec|odeº
Couçe|o|·B|ur d|ºe+ºe, +|ºo |uoWu +º p|ç·
reu|ed pu|pu||c ||c|euo|d de|r+||||º o| pu|·
pu|+ p|çreu|oº+ c||ou|c+
||c|eu +u|euº , +|ºo |uoWu +º ||c|eu pu|pu||cuº.
C||u|c+||,, e+c| eu|||, º|oWº |eceu| p|upo|u| c+,·
euue peppe|-co|o|ed |ero|||+çeº +ººoc|+|ed
W||| o|de| |ero|||+çeº +ud |eroº|de||u depoº|·
||ou. C+p|||+||||º ||º|o|oç|c+||,.
||| +|e º|çu|||c+u| ou|, || ||e, +|e + coºre||c
couce|u |o ||e p+||eu|, ||e, +|e |rpo||+u| |e·
c+uºe ||e, +|e o||eu r|º|+|eu +º r+u||eº|+||ouº
o| .+ºcu||||º o| |||or|oc,|opeu|+.
',¤¤¤,¤ . C+p|||+||||º o| uu|uoWu c+uºe.
|C|·9. 109.¹

|C|·¹0. |3¹.1
FICM£NI£0 FükFükIC 0£kMAI0S£S (FF0)
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset 30-60 yeais; uncommon in
childien.
Sex Moie common in males.
£tìo|o¿y Unknown. Piimaiy piocess believed
to be cell-mediated immune injuiy with subse-
quent vasculai damage and eiythiocyte extiava-
sation. Othei etiologic factois: piessuie, tiauma,
diugs (acetaminophen, ampicillin-caibiomal,
diuietics, mepiobamate, nonsteioidal anti-
inflammatoiy diugs, zomepiiac sodium).
0oset aod 0uratìoo Insidious, slow to evolve
except diug-induced vaiiant, which may develop
iapidly and be moie geneialized in distiibution.
Peisists foi months to yeais. Most diug-induced
puipuias iesolve moie quickly aftei discontinua-
tion of the diug. Usually asymptomatic but may
be mildly piuiitic. It can be quite cosmetically
disfiguiing. Any piuiitus piobably ielated to dei-
matitis (asteatotic, atopic, oi stasis) on lowei legs.
CIINICAI MANIF£SIAII0N
Schamber¿ 0ìsease Disciete clusteis of
pinhead-sized ied macules and baiely palpa-
ble papules become confluent, coalescing into
patches (Fig. 7-16). Diascopy ieveals pinpoint
hemoiihages (hence the teim ¡ur¡ura ). New
lesions aie ied; oldei lesions tan to biown,
iepiesenting degiadation of extiavasated eiyth-
iocytes with the foimation of hemosideiin.
Oveiall coloi impiession: ieddish biown, °cay-
enne peppei" (Fig. 7-16). Lowei extiemities
(especially pietibial and on ankles) but may
extend pioximally to lowei tiunk and to uppei
extiemities. Usually bilateial but may be unilat-
eial. Uncommonly, geneialized.
Majocchì 0ìsease Essentially an annulai foim
of Schambeig disease with telangiectasias
(Fig. 7-17). An aicifoim vaiiant has also been
desciibed.
Cou¿erot-8|um 0ìsease Lichenoid papules,
plaques, macules in association with lesions of
Schambeig disease.
Iìcheo Aureus Solitaiy oi few patches oi
plaques, iust-coloied, puiple, oi golden, aiising
on the extiemities oi tiunk.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Epideimal involvement
vaiies, but deimal pathology (capillaiitis) with
extiavasation of eiythiocytes, hemosideiin
pigment-laden maciophages (moie extensive
in lichen auieus), mild peiivasculai and intei-
stitial lymphohistiocytic infiltiate in ieticulai
deimis is common to all. Immunofluoiescence
is vaiiable and nonspecific.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Noopa|pab|e Furpura Chionic venous insuf-
ficiency with clotting abnoimalities, gluco-
coiticoid usage, cutaneous T cell lymphoma;
dyspioteinemias, nummulai eczema, old fixed
diug eiuption, paiapsoiiasis, poikilodeima
vasculaie atiophicans, piimaiy amyloidosis,
scuivy, senile puipuia, stasis deimatitis, thiom-
bocytopenia, tiauma.
Fa|pab|e Furpura Leukocytoclastic vasculitis.
Thiombocytopenic puipuia.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 145
C0ükS£
Chionic (months to yeais), slow to evolve
and iesolve; spontaneous iesolution has
occuiied. In lesions of long standing,
hemosideiin deposits iesolve veiy slowly
(months to yeais). Almost all cases due to
diugs cleai within months aftei discon-
tinuation of the offending agent.
MANAC£M£NI
Symptomatìc Long-standing lesions aie
cosmetically disfiguiing, and patients
may choose to tieat these lesions. Topical
low- and middle-potency glucocoiticoid
piepaiations may inhibit new puipuiic
lesions. Systemic tetiacycline oi mino-
cycline (50 mg twice daily) aie effective.
PUVA is effective in seveie foims. Su¡-
¡orìí·e sìot|íngs reguíreJ ín a|| [orms.
FICük£ T-16 Fì¿meoted purpurìc der-
matosìs: Schamber¿ dìsease \u|||p|e
d|ºc|e|e +ud cou||ueu| uoup+|p+||e, uou·
||+uc||uç pu|pu||c |eº|ouº ou ||e |eç. Acu|e
r|c|o|ero|||+çeº |eºo|.e W||| depoº|||ou o|
|eroº|de||u, c|e+||uç + ||oWu peppe|ed º|+|u.
FICük£ T-1T Fì¿meoted purpurìc
dermatosìs: Majocchì dìsease \u|||p|e
uoup+|p+||e, uou||+uc||uç pu|pu||c |eº|ouº
+||+uçed |u +uuu|+| cou||çu|+||ouº. |o|e. d|º||ç·
u||uç d+|| ||oWu d|ºco|o|+||ou o| o|d |eº|ouº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 146
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset Adolescents and young adults.
Sex Moie common in males than females.
£tìo|o¿y Unknown.
CIINICAI MANIF£SIAII0N
Lesions tend to appeai in ciops ovei a peiiod of
weeks oi months. Uncommonly, patients with
an acute onset of the disoidei may have symp-
toms of an acute infection with fevei, malaise,
and headache. Cutaneous lesions aie usually
asymptomatic but may be piuiitic oi sensitive
to touch. Lesions may heal with significant
scaiiing and postinflammatoiy pigmentation.
Especially in that it occuis in adolescents and
young adults.
Skìo Iesìoos Initially, iandomly distiibuted,
biight-ied edematous papules (i.e., lichenoides),
less commonly vesicles, which undeigo cential
neciosis with hemoiihagic ciusting (i.e.,
vaiiolifoimis, hence the designation PLEVÀ )
(Fig. 7-18 À and B ). In the chionic foim (PLC),
scaling papules of ieddish-biown coloi and a
cential mica-like scale aie seen (Fig. 7-18 C ).
Postinflammatoiy hypo- oi hypeipigmentation
often piesent aftei lesions iesolve. PLEVA may
heal with depiessed oi elevated scais.
DIstrIhutIvn Randomly aiianged, most com-
monly on tiunk, pioximal extiemities but also
geneialized, including palms and soles.
0ra| aod Ceoìta| Mucosa Inflammatoiy pa-
pules and neciotic lesions may occui.
|| |º +u e|up||ou o| uu|uoWu e||o|oç,, c|+|+c|e|·
|/ed c||u|c+||, |, ºucceºº|.e c|opº o| + W|de |+uçe
o| ro|p|o|oç|c |eº|ouº.
C|+ºº|||ed |u|o +u +cu|e |o|r, p||,||+º|º ||c|euo|deº
e| .+||o|||o|r|º +cu|+ (||E\A, \uc|+·n+|e|r+uu
d|ºe+ºe), +ud + c||ou|c |o|r, p||,||+º|º
||c|euo|deº c||ou|c+ (||C, çu||+|e p+|+pºo||+º|º
o| lu||uº|e|ç).
noWe.e|, roº| p+||eu|º |+.e |eº|ouº o| ||E\A
+ud ||C º|ru||+ueouº|,.
||E\A |º |rpo||+u| |ec+uºe || c+u |e r|º|+|eu |o|
|,rp|or+|o|d p+pu|oº|º (ºee 'ec||ou 20).
',¤¤¤,¤ Cu||+|e p+|+pºo||+º|º.
FII¥kIASIS IICh£N0I0£S (ACüI£ AN0 Chk0NIC) (FI)
|C|·9 . o9o.2

|C|·¹0 . |+¹.0/|+¹.¹
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y E¡íJermís : spongiosis, ke-
iatinocyte neciosis, vesiculation, ulceiation;
exocytosis oi eiythiocytes within epideimis.
Dermís : Edema, chionic inflammatoiy cell infil-
tiate in wedge shape extending to deep ieticulai
deimis; hemoiihage; vessels congested with
blood; endothelial cells swollen.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical diagnosis is confiimed by skin biopsy.
Diffeiential diagnosis: vaiicella, guttate psoiia-
sis, lymphomatoid papulosis.
C0ükS£ AN0 Fk0CN0SIS
New lesions appeai in successive ciops.
PLC tends to iesolve spontaneously aftei
6-12 months. In some cases, ielapses aftei many
months oi yeais.
MANAC£M£NI
Most patients do not iequiie any theiapeutic
inteivention. Oial eiythiomycin and tetiacy-
cline aie iepoited to be effective in some cases.
Ultiaviolet iadiation (whethei natuial sunlight
oi bioad-band UVB), 311-nm UVB, and PUVA
aie the tieatments of choice if oial antibiotics
fail aftei a 2-week tiial.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 14T



FICük£ T-18 Fìtyrìasìs |ìcheooìdes et varìo|ìIormìs acuta (FI£vA)  k+udor|, d|º||||u|ed |ed p+p·
u|eº o| d|||e|eu| º|/e, ºore o| W||c| º|oW |ero|||+ç|c c|uº||uç. |u |||º 5·,e+|·o|d c|||d ||e e|up||ou +ppe+|ed |u
c|opº o.e| + pe||od o| ¹0 d+,º.  FI£vA |eº|ouº |u + !3·,e+|·o|d |udoueº|+u r+u. |eº|ouº +|e ro|e |,pe|p|ç·
reu|ed +ud ||e|e |º couº|de|+||e ºc+||uç +ud c|uº||uç.  Fìtyrìasìs |ìcheooìds chrooìca (FIC) ||ºc|e|e p+pu|eº
W||| ||ue r|c+·|||e ºc+|eº W||c| |ecore ro|e .|º|||e +||e| º||ç|| ºc|+p|uç. |u cou||+º| |o ||E\A (||ç. 1·¹3A +ud B),
||e|e |º uo |ero|||+ç|c c|uº||uç
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 148
£FI0£MI0I0C¥
A¿e oI 0oset 50% undei 20 yeais.
Sex Moie fiequent in males than in females.
£II0I0C¥
A cutaneous ieaction to a vaiiety of antigenic
stimuli, most commonly to heipes simplex.
IoIectìoo Especially following heipes simplex,
Myto¡|asma .
0ru¿s Sulfonamides, phenytoin, baibituiates,
phenylbutazone, penicillin, allopuiinol.
Idìopathìc Piobably also due to undetected
heipes simplex oi M yto¡|asma .
CIINICAI MANIF£SIAII0N
Evolution of lesions ovei seveial days. May have
histoiy of piioi EM. May be piuiitic oi pain-
ful, paiticulaily mouth lesions. In seveie foims
constitutional symptoms such as fevei, weak-
ness, malaise.
Skìo Iesìoos Lesions may develop ovei 10
days. Macule papule (1-2 cm) vesicles
and bullae in the centei of the papule; (Fig.
7-19). Dull ied. Irís oi ìargeì|í|e |esíons iesult
and aie typical (Figs. 7-19 and 7-20). Localized
to hands and face oi geneialized (Figs. 7-21 and
7-22). Bilateial and often symmetiic.
SItes v] PredI|ectIvn Doisa of hands, palms,
and soles; foieaims; feet; face (Figs. 7-20 and
7-21); elbows and knees; penis (50%) and vulva
(Image 7-3).
Mucous Membraoes Eiosions with fibiin
membianes; occasionally ulceiations: lips
(Fig. 7-20), oiophaiynx, nasal, conjunctival
(Fig. 7-21), vulvai, anal.
0ther 0r¿aos Eyes, with coineal ulceis, ante-
iioi uveitis.
A corrou |e+c||ou p+||e|u o| ||ood .eººe|º |u
||e de|r|º W||| ºecoud+|, ep|de|r+| c|+uçeº.
\+u||eº|º c||u|c+||, +º c|+|+c|e||º||c e|,||er+|ouº
|||º·º|+ped p+pu|+| +ud .eº|cu|o|u||ouº |eº|ouº.
I,p|c+||, |u.o|.|uç ||e e\||er|||eº (eºpec|+||, ||e
p+|rº +ud ºo|eº) +ud ||e rucouº rer||+ueº.
Beu|çu cou|ºe W||| ||equeu| |ecu||euceº.
\oº| c+ºeº |e|+|ed |o |e|peº º|rp|e\ .||uº (n'\)
|u|ec||ou
kecu||euceº c+u |e p|e.eu|ed |, |ouç·|e|r +u||·
n'\ red|c+||ou.
|C|·9 . o95.¹

|C|·¹0 . |5¹
£k¥Ih£MA MüIIIF0kM£ S¥N0k0M£ (£M)
C0ükS£
Mì|d Forms (£M Mìoor) Little oi no mucous
membiane involvement; vesicles but no bullae
oi systemic symptoms. Eiuption usually con-
fined to extiemities, face, classic taiget lesions
(Figs. 7-19 and 7-20). Recuiient EM minoi is
usually associated with an outbieak of heipes
simplex pieceding it by seveial days.
Severe Forms (£M Major) Most often occuis as
a diug ieaction, always with mucous membiane
involvement; seveie, extensive, tendency to be-
come confluent and bullous, positive Nikolsky
sign in eiythematous lesions (Fig. 7-21). Sys-
temic symptoms: fevei, piostiation. Cheilitis
and stomatitis inteifeie with eating; vulvitis and
balanitis with mictuiition. Conjunctivitis can
lead to keiatitis and ulceiation; lesions also in
phaiynx and laiynx.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Inflammation chaiactei-
ized by peiivasculai mononucleai infiltiate,
edema of the uppei deimis; apoptosis of ke-
iatinocytes with focal epideimal neciosis and
subepideimal bulla foimation. In seveie cases,
complete neciosis of epideimis as in toxic epi-
deimal neciolysis. (See Section 8.)
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
The taiget-like lesion and the symmetiy aie
quite typical, and the diagnosis is not difficult.
Acute £xaothematìc £ruptìoos Diug eiuption,
psoiiasis, secondaiy syphilis, uiticaiia, gen-
eialized Sweet syndiome. Mucous membiane
lesions may piesent a difficult diffeiential di-
agnosis: bullous diseases, fixed diug eiuption,
acute lupus eiythematosus, piimaiy heipetic
gingivostomatitis.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 149
FICük£ T-19 £rythema mu|tìIorme |||º o| |+|çe| |eº|ouº ou ||e |oWe| +|r o| + ¹5·,e+|·o|d. I|eºe +|e
º|+|p|, de||ued ||+| p+pu|eº W||| + ceu||+| .eº|c|e.
IMAC£ T-3 £rythema mu|tìIorme p|ed||ec||ou º||eº +ud d|º||||u||ou.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 150
FICük£ T-20 £rythema mu|tìIorme: mìoor \u|||p|e, cou||ueu|, |+|çe|·|||e p+pu|eº ou ||e |+ce o| +
¹2·,e+|·o|d |o,. I|e |+|çe| ro|p|o|oç, o| ||e |eº|ouº |º |eº| ºeeu ou ||e ||pº.
FICük£ T-21 £rythema mu|tì-
Iorme: major E|,||er+|ouº, cou||u·
eu|, |+|çe|·|||e p+pu|eº, p|+queº, +ud
e|oº|ouº ou ||e ||uu|, ||e +|rº, +ud
||e |+ce. |+c|+| |eº|ouº +|e e|oº|.e +ud
c|uº|ed. I|e|e |º e|oº|.e c|e|||||º |ud|c+|·
|uç rucoº+| |u.o|.ereu|, +ud ||e|e |º
coujuuc||.|||º.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 151
FICük£ T-22 £rythema mu|tìIorme: major \u|||p|e, |+|çe| |eº|ouº |+.e co+|eºced, +ud e|oº|ouº W|||
de.e|op. I||º p+||eu| |+d |e.e| +ud rucoº+| |u.o|.ereu| o| rou||, coujuuc||.+, +ud çeu||+||+
MANAC£M£NI
Freveotìoo Contiol of heipes simplex using
oial valacyclovii oi famciclovii may pievent
development of iecuiient EM.
C|ucocortìcoìds In seveiely ill patients, sys-
temic glucocoiticoids aie usually given (pied-
nisone, 50-80 mg/d in divided doses, quickly
tapeied), but theii effectiveness has not been
established by contiolled studies.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 152
£FI0£MI0I0C¥ AN0 £II0I0C¥
The most common type of panniculitis, with a
peak incidence at 20-30 yeais, but any age may
be affected. Thiee to six times moie common
on females than males.
£tìo|o¿y EN is not a disease but a cutaneous
ieaction pattein to vaiious etiologic agents.
Etiologic associations include infections, diugs,
and othei inflammatoiy/gianulomatous dis-
eases, notably saicoidosis (Table 7-2).
CIINICAI MANIF£SIAII0N
Painful, tendei lesions, usually of a few days`
duiation, aie accompanied by fevei, malaise,
and aithialgia (50%), most fiequently of ankle
joints. Othei symptoms, depending on etiology.
Skìo Iesìoos Induiated, veiy tendei nodules
(3-20 cm), not shaiply maiginated (Fig. 7-23),
deep seated in the subcutaneous fat, mostly
on the anteiioi lowei legs, bilateial but not
symmetiic. Nodules aie biight to deep ied and
aie appieciated as such only upon palpation.
The teim eryì|ema noJosum best desciibes
the skin lesions: ì|ey |oo| |í|e eryì|ema |uì
[ee| |í|e noJu|es (Fig. 7-23). Lesions aie oval,
iound, aicifoim; as they age, they become
violaceous, biownish, yellowish, gieen, like
iesolving hematomas. Lesions may also occui
on knees and aims but only iaiely on the face
and on the neck.
IA80kAI0k¥ £XAMINAII0NS
hemato|o¿y Elevated eiythiocyte sedimenta-
tion iate (ESR), C-ieactive piotein elevated,
leukocytosis.
E| |º +u |rpo||+u| +ud corrou +cu|e |u||+rr+·
|o|,/|rruuo|oç|c |e+c||ou p+||e|u o| ||e ºu|cu|+·
ueouº |+|.
C|+|+c|e||/ed |, ||e +ppe+|+uce o| p+|u|u| uod·
u|eº ou ||e |oWe| |eçº.
|eº|ouº +|e |||ç|| |ed +ud ||+| |u| uodu|+| upou
p+|p+||ou.
0||eu |e.e| +ud +|||||||º.
\u|||p|e +ud d|.e|ºe e||o|oç|eº.
£k¥Ih£MA N000SüM (£N) S¥N0k0M£
|C|·9 . o95.2

|C|·¹0 . |52
8acterìa| Cu|ture Cultuie thioat foi gioup A
-hemolytic stieptococcus, stool foi Yersínía .
Ima¿ìo¿ Radiologic examination of the chest
and gallium scan aie impoitant to iule out oi
piove saicoidosis.
0ermatopatho|o¿y Acute (polymoiphonu-
cleai) and chionic (gianulomatous) inflamma-
tion in the panniculus and aiound blood vessels
in the septum and adjacent fat. It is a septal
panniculitis.
C0ükS£
Spontaneous iesolution occuis in 6 weeks,
with new lesions eiupting duiing that time.
Couise depends on the etiology. Lesions nevei
bieak down oi ulceiate and heal without
scaiiing.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Diagnosis iests on clinical ciiteiia, may be sup-
poited by histopathology. Diffeiential diagnosis
includes all othei foims of panniculitis, pan-
aiteiitis nodosa, nodulai vasculitis, pietibial
myxedema, nonulceiated gumma, and lym-
phoma.
MANAC£M£NI
Symptomatìc Bed iest oi compiessive band-
ages (lowei legs), wet diessings.
Aotì-IoI|ammatory Ireatmeot Salicylates, non-
steioidal anti-inflammatoiy diugs. Systemic
glucocoiticoids-iesponse is iapid, but theii
use is indicated only when the etiology is
known (and infectious agents aie excluded).
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 153
IA8I£ T-2 Causes of Erythema Nodosum

IoIectìoos 0ther
8acterìa| 0ru¿s
'||ep|ococc+| |u|ec||ouº, |u|e|cu|oº|º, ,e|º|u|oº|º 'u||ou+r|deº, ||or|deº +ud |od|deº,
0||e|. 'c|¤¤¤-||c Cc¤¤,|¤|c:|-· '|·¸-||c, 0|+| cou||+cep||.eº
||uce||oº|º, pº|||+coº|º, !,:¤¤|cº¤c 0||e|. r|uoc,c||ue, ço|d º+||º, peu|c||||u,
º+||c,|+|eº
Fuo¿a| Ma|ì¿oaocìes
Cocc|d|o|dor,coº|º, ||+º|or,coº|º, nodç||u +ud uou·nodç||u |,rp|or+,
||º|op|+ºroº|º, ºpo|o|||c|oº|º, |eu|er|+, |eu+| ce|| c+|c|uor+
de|r+|op|,|oº|º
vìra| 0ther
|u|ec||ouº rououuc|eoº|º, |ep+||||º B, o||, '+|co|doº|º
|e|peº º|rp|e\ |u||+rr+|o|, |oWe| d|ºe+ºe. u|ce|+||.e
0ther co||||º, C|o|u d|ºe+ºe
Are||+º|º, ç|+|d|+º|º, +ºc+||+º|º Be|(e| d|ºe+ºe
+
|o| + ro|e corp|e|e ||º| o| e||o|oç|c |+c|o|º |u E|, ºee | kequeu+ e| +|, |u K, wo||| e| +| (edº,). |·|c¤c|··:|'º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-,
1|| ed, |eW \o||, \cC|+W·n|||, 2003, p 5o9-535
FICük£ T-23 £rythema oodosum |udu|+|ed, .e|, |eude|, |u||+rr+|o|, uodu|eº roº||, |u ||e p|e||||+|
|eç|ou. |eº|ouº +|e ºeeu +º |ed, |||·de||ued e|,||er+º |u| p+|p+|ed +º deep·ºe+|ed uodu|eº, |euce ||e deº|çu+·
||ou. |u |||º +9·,e+|·o|d |er+|e ||e|e W+º +|ºo |e.e| +ud +|||||||º o| ||e +u||e jo|u|º |o||oW|uç +u uppe| |eºp||+|o|,
||+c| |u|ec||ou. I|e |||o+| cu||u|eº ,|e|ded ·|ero|,||c º||ep|ococc|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 154
|+uu|cu||||º |º ||e |e|r uºed |o deºc|||e d|ºe+ºeº
W|e|e ||e r+jo| |ocuº o| |u||+rr+||ou |º |u ||e
ºu|cu|+ueouº ||ººue. |u çeue|+|, p+uu|cu||||º p|e·
ºeu|º +º +u e|,||er+|ouº o| .|o|+ceouº uodu|e |u
||e ºu|cu|+ueouº |+| ||+| r+, |e |eude| o| uo|,
||+| r+, u|ce|+|e o| |e+| W|||ou| ºc+|||uç, +ud
||+| r+, |e ºo|| o| |+|d ou p+|p+||ou. I|uº, ||e
|e|r ¤c¤¤·:±|·|·º deºc|||eº + W|de ºpec||ur o|
d|ºe+ºe r+u||eº|+||ouº, +|||ouç| d|+çuoº||c c|ueº
c+u |e de||.ed ||or ||e ||º|o|,, d|º||||u||ou, o|
c|+|+c|e||º||cº o| ||e |eº|ouº.
Au +ccu|+|e d|+çuoº|º |equ||eº +u +rp|e deep
º||u ||opº, ||+| º|ou|d |e+c| doWu |o o| e.eu
|e,oud ||e |+ºc|+. I|e p+uu|cu||||deº +|e c|+ºº|·
||ed ||º|o|oç|c+||, +º |o|u|+| o| ºep|+|, depeud|uç
ou W|e|e ||e d|ºe+ºe p|oceºº |eç|uº. noWe.e|,
+ º|+|p d|º||uc||ou |e|Weeu ºep|+| +ud |o|u|+| |º
o||eu uo| poºº|||e. |+uu|cu||||º r+, +|ºo |e +ººo·
c|+|ed W||| .+ºcu||||º, |u| |u roº| c+ºeº ||e|e |º uo
.+ºcu||||º. A º|rp||||ed c|+ºº|||c+||ou o| p+uu|cu||||º
|º ç|.eu |u I+||e 1·!.
0u|, |d|op+|||c |o|u|+| p+uu|cu||||º (||e|||e|·
we|e|·C|||º||+u d|ºe+ºe), p+uc|e+||c p+uu|cu||·
||º, +ud
¹
+u||||,pº|u·de||c|euc, p+uu|cu||||º +|e
|||e||, d|ºcuººed |e|e. 0||e| d|ºe+ºeº |u W||c|
p+uu|cu||||º occu|º +|e |e|e||ed |o |u ||e |+||e.
¹
|!·¤¤c||·: |¤|±|c· ¤c¤¤·:±|·|·º (|||) , W||c| occu|º
p|edor|u+u||, |u |er+|eº !0-o0 ,e+|º o| +çe, |u|
c+u +|ºo occu| |u c|||d|ood, r+u||eº|º +º c|opº o|
ºu|cu|+ueouº |u||+rr+|o|, +ud |eude| uodu|eº,
p||r+|||, ou ||e |oWe| e\||er|||eº |u| +|ºo ou ||e
||uu| +ud e|ºeW|e|e (||ç. 1·2+). 0cc+º|ou+||,,
|eº|ouº c+u ||e+| doWu, d|ºc|+|ç|uç +u o||,
,e||oW·||oWu ||qu|d, çeue|+||, +ccorp+u|ed |,
r+|+|ºe, |+||çue, |e.e|, +||||+|ç|+, +ud r,+|ç|+.
I|e|e r+, |e |oc+| uec|oº|º |u ||e |u||+.|ºce|+|
+ud pe||.|ºce|+| |+| o| |u|e|u+| o|ç+uº, |uc|ud|uç
||e reºeu|e||c +ud oreu|+| |+|, pe||c+|d|ur,
+ud p|eu|+. 0|ç+u |u.o|.ereu| r+, p|eºeu|
+º |ep+|oreç+|,, +|dor|u+| p+|u, u+uºe+, +ud
.or|||uç.
I|e e||o|oç, |º uu|uoWu. |¤|- r+u, c+ºeº o| |||
|+.e |eeu |ec|+ºº|||ed +º o||e| r+u||eº|+||ouº o|
|o|u|+| p+uu|cu||||º +ud ||e|e |º + uoW ç|oW|uç
dou|| ||+| ||| e\|º|º +º +u eu|||,.
|c¤:·-c|·: ¤c¤¤·:±|·|·º |º c|+|+c|e||/ed c||u|c+||,
|, p+|u|u| e|,||er+|ouº uodu|eº +ud p|+queº ||+|
r+, ||uc|u+|e +ud occu| +| +u, º||e, W||| + p|ed|·
|ec||ou |o| +|doreu, |u||oc|º, |eçº (||ç. 1·25).
||equeu||, +ccorp+u|ed |, +|||||||º +ud po|,ºe·
|oº|||º. Aººoc|+|ed W||| e|||e| p+uc|e+||||º o| p+u·
c|e+||c c+|c|uor+. || +||ec|º r|dd|e·+çed |o e|de||,
|ud|.|du+|º, r+|eº ro|e o||eu ||+u |er+|eº. n|º·
|o|,. +|co|o||ºr, +|dor|u+| p+|u, We|ç|| |oºº, o|
|eceu|·ouºe| d|+|e|eº re||||uº. '||u ||opº, |e.e+|º
|o|u|+| p+uu|cu||||º, +ud +||e| ||opº, ||que||ed |+|
d|+|uº ||or ||e ||opº, º||e. Ceue|+| e\+r|u+||ou
r+, |e.e+| p|eu|+| e||uº|ou, +ºc||eº, +ud +|||||||º,
p+|||cu|+||, o| ||e +u||eº.
|+|o|+|o|,. eoº|uop||||+, |,pe|||p+ºer|+, |,pe|·
+r,|+ºer|+, +ud |uc|e+ºed e\c|e||ou o| +r,|+ºe
+ud/o| ||p+ºe |u ||e u||ue. I|e p+||op|,º|o|oç,
|º p|o|+||, + ||e+|doWu o| ºu|cu|+ueouº |+|
c+uºed |, eu/,reº (+r,|+ºe, ||,pº|u, ||p+ºe)
|e|e+ºed |u|o ||e c||cu|+||ou ||or + d|ºe+ºed p+u·
c|e+º. I|e cou|ºe +ud p|oçuoº|º depeud ou ||e
|,pe o| p+uc|e+||c d|ºe+ºe. I|e+|reu| |º d||ec|ed
+| ||e uude||,|uç p+uc|e+||c d|ºo|de|.
¹
1¤|·|·,¤º·¤·!-|·:·-¤:, ¤c¤¤·:±|·|·º |º +|ºo
c|+|+c|e||/ed |, |ecu||eu| |eude|, e|,||er+|ouº,
ºu|cu|+ueouº uodu|eº |+uç|uç ||or ¹ |o 5 cr
+ud |oc+|ed p|edor|u+u||, ou ||e ||uu| +ud ||e
p|o\|r+| e\||er|||eº, .e|, ruc| |||e ||oºe º|oWu
|u ||ç. 1·25. |odu|eº ||e+| doWu +ud d|ºc|+|çe
+ c|e+| ºe|ouº o| o||, ||u|d. ||+çuoº|º |º ºu|º|+u||·
+|ed |, + dec|e+ºe |u ||e |e.e| o| ºe|ur
¹
+u|||·
|,pº|u, +ud ||e+|reu| couº|º|º o| o|+| d+pºoue |u
doºeº up |o 200 rç/d. I|e |u||+.euouº |u|uº|ou
o| |ur+u
¹
·p|o|e|u+ºe |u|||||o| couceu||+|e |+º
|eeu º|oWu |o |e .e|, e||ec||.e.
¹
I|e |e+de| |º +|ºo |e|e||ed |o |.kequeu+ e| +|, |u
K wo||| e| +| (edº). |·|c¤c|··:|'º |-·¤c|¤|¤¸, ·¤
C-¤-·c| !-!·:·¤- , 1|| ed. |eW \o||, \cC|+W·n|||,
2003, p 5o9.
0Ih£k FANNICüIIII0£S |C|·9 . 129.!

|C|·¹0 . \19.!
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 155
IA8I£ T-3 Simp|ified C|assification of Pannicu|itis
Lob0|ar Paoo|c0||t|s Septa| Paoo|c0||t|s
|eou+|+| 'c|e|er+ ueou+|o|ur,
ueou+|+| ºu|cu|+ueouº |+| uec|oº|º
||,º|c+| Co|d, ||+ur+
||uçº |oº|º|e|o|d p+uu|cu||||º E|,||er+ uodoºur
|d|op+|||c |d|op+|||c |o|u|+| p+uu|cu||||º Eoº|uop||||c |+ºc||||º
(||e|||e|·we|e|·C|||º||+u) º,ud|ore Eoº|uop||||+ r,+|ç|+ º,ud|ore
|+uc|e+||c w||| p+uc|e+||||º o| c+|c|uor+ o| ||e
p+uc|e+º
|+uu|cu||||º W||| o||e| |upuº e|,||er+|oºuº, º+|co|doº|º, 'c|e|ode|r+
º,º|er|c d|ºe+ºe |,rp|or+, ||º||oc,||c c,|op|+ç|c
p+uu|cu||||º
w||| .+ºcu||||º |odu|+| .+ºcu||||º I||or|op||e||||º,
p+u+||e||||º uodoº+
\e|+|o||c de||c|euc,
¹
·+u||||,pº|u de||c|euc,
FICük£ T-24 Idìopathìc |obu|ar paooìcu|ìtìs ìo a 5-year-o|d boy A|||ouç| |eº|ouº uºu+||, +||ºe ou
||e |oWe| e\||er|||eº, ||e, r+, r+u||eº| +º c|opº o| ºu|cu|+ueouº |u||+rr+|o|, +ud |eude| uodu|eº +|ºo ou
||e ||uu| +ud ou ||e |+ce. uºu+||, occu|||uç |u |er+|eº !0 |o o0 ,e+|º o|d, || c+u +|ºo +ppe+| |u c|||d|eu, +º
|º ||e c+ºe W||| |||º |o,. |o e||o|oç|c +çeu| W+º |ouud. I|e|e W+º |ep+|oreç+|,, +ud +|dor|u+| p+|u. I|e
p+||eu| |eºpouded |o ç|ucoco|||co|dº |u| |+d |ecu||euceº
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 156
FICük£ T-25 Faocreatìc
paooìcu|ìtìs I|e|e +|e ru|·
||p|e, p+|u|u|, e|,||er+|ouº
uodu|eº +ud p|+queº ||+| ||uc|u·
+|e ou ||e |oWe| e\||er|||eº, |u|
º|r||+| |eº|ouº We|e +|ºo |ouud
ou ||e ||uu| +ud ou ||e |u||oc|º
|C |º + |+p|d|, e.o|.|uç, |d|op+|||c, c||ou|c, +ud
ºe.e|e|, de|||||+||uç º||u d|ºe+ºe.
|| |º c|+|+c|e||/ed |, ueu||op||||c |u|||||+||ou +ud
deº||uc||ou o| ||ººue.
|| occu|º roº| corrou|, |u +ººoc|+||ou W||| +
º,º|er|c d|ºe+ºe, eºpec|+||, c||ou|c u|ce|+||.e
co||||º.
|| +|ºo occu|º W||| +|||||||º, |er+|o|oç|c d,ºc|+·
º|+º, +ud r+||çu+uc, |u| r+, occu| +|ºo +|oue.
|| |º c|+|+c|e||/ed |, ||e p|eºeuce o| |||eçu|+|,
|oçç,, ||ue·|ed u|ce|º W||| uude|r|ued |o|de|º
ºu||ouud|uç pu|u|eu| uec|o||c |+ºeº.
I|e|e |º uo |+|o|+|o|, |eº| ||+| eº|+|||º|eº ||e
d|+çuoº|º.
I|e r+|uº|+,º o| ||e+|reu| +|e |rruuoºupp|eº·
º|.e o| rodu|+||uç +çeu|º.
F¥00£kMA CANCk£N0SüM (FC) |C|·9 . o3o.0¹

|C|·¹0 . |33
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 15T
£FI0£MI0I0C¥
Raie, pievalence unknown. All age gioups af-
fected with a peak between 40 and 60 yeais.
Slight piepondeiance of females.
£II0I0C¥ AN0 FAIh0C£N£SIS
Unknown. Although called pyodeima, it does
not have a miciobial etiology. PG is counted
among the neutiophilic deimatoses because
of the massive neutiophilic infiltiates within
the skin.
CIINICAI MANIF£SIAII0N
Iwo Iypes Àtuìe. acute onset with painful
hemoiihagic pustule oi painful nodule eithei
de novo oi aftei minimal tiauma. C|ronít. slow
piogiession with gianulation and hypeikeiato-
sis. Less painful.
Skìo Iesìoos Àtuìe. Supeificial hemoiihagic
pustule suiiounded by eiythematous halo;
veiy painful (Fig. 7-26 À ). Bieakdown occuis
with ulcei foimation, wheieby ulcei boideis
aie dusky-ied oi puiple, iiiegulai and
iaised, undeimined, boggy with peifoiations
that diain pus (Fig. 7-26 B ). The base of the
ulcei is puiulent with hemoiihagic exudate,
paitially coveied by neciotic eschai (Fig.
7-27), with oi without gianulation tissue.
Pustules both at the advancing boidei and
in the ulcei base; a halo of eiythema spieads
centiifugally at the advancing edge of the
ulcei (Figs. 7-26 B and 7-27). C|ronít. lesions
may slowly piogiess, giazing ovei laige aieas
of the body and exhibiting massive gianulation
within the ulcei fiom the outset (Fig.
7-28) and ciusting and even hypeikeiatosis
on the maigins (Fig. 7-29, page 160).
Lesions aie usually solitaiy but may be
multiple and foim clusteis that coalesce.
Most common sites: lowei extiemities
(Figs. 7-26 B and 7-29) > buttocks > abdomen
(Fig. 7-27) > face (Fig. 7-28). Healing of ulceis
iesults in thin atiophic ciibiifoim scais.
Mucous Membraoes Raiel y, aphthous
stomatitis-like lesions; massive ulceiation of
oial mucosa and conjuctivae.
Ceoera| £xamìoatìoo
Patient may appeai ill.
Assocìated Systemìc 0ìseases
Up to 50% of cases occui without associated
disease. Remaindei of cases associated with
laige- and small-bowel disease (Ciohn disease,
ulceiative colitis), diveiticulosis (diveiticulitis),
aithiitis, paiapioteinemia and myeloma, leuke-
mia, active chionic hepatitis, Behçet syndiome.
IA80kAI0k¥ £XAMINAII0NS
Theie is no single diagnostic test.
£Sk Vaiiably elevated.
0ermatopatho|o¿y Not diagnostic. Neu-
tiophilic inflammation with abscess foimation
and neciosis.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical findings plus histoiy and couise; con-
fiimed by nonspecific deimatopathology show-
ing neutiophilic inflammation with abscesses
and neciosis. Diffeiential diagnosis: ecthyma
and ecthyma gangienosum, atypical mycobac-
teiial infection, clostiidial infection, deep my-
coses, amebiasis, leishmaniasis, biomodeima,
pemphigus vegetans, stasis ulceis, Wegenei
gianulomatosis.
C0ükS£ AN0 Fk0CN0SIS
Untieated, couise may last months to yeais,
but spontaneous healing can occui. Ulceiation
may extend iapidly within a few days oi slowly.
Healing may occui centially with peiipheial ex-
tension. New ulceis may appeai as oldei lesions
iesolve. Patheigy, i.e., slight tiauma initiating
new PG lesion, noted at sites of minoi tiauma,
biopsy, oi needle sticks.
MANAC£M£NI
Wìth Assocìated üoder|yìo¿ 0ìsease Tieat un-
deilying disease.
For FC High doses of oial glucocoiticoids
oi IV glucocoiticoid pulse theiapy (1-2 g/d
piednisolone) may be iequiied. Sulfasalazine
(paiticulaily in cases associated with Ciohn
disease), sulfones, cyclospoiine, and, moie ie-
cently, infliximab, etaneicept, adalimumab have
been shown to be effective in uncontiolled
studies.
Iopìca| In singulai lesion, topical taciolimus
ointment oi intialesional tiiamcinolone.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 158
FICük£ T-26 Fyoderma ¿ao¿reoo-
sum  I|e |u|||+| |eº|ou |º + |ero|·
||+ç|c uou|o|||cu|+| puº|u|e ºu||ouuded |,
+u e|,||er+|ouº |+|o +ud |º .e|, p+|u|u|.
 |eº|ouº |+p|d|, eu|+|çe +ud ||e+| doWu
ceu||+||, +º º|oWu |e|e. |o|e. e|,||er+·
|ouº |+|o +|ouud ||e |ud|.|du+| |eº|ouº.
I|e|e |º +ccorp+u,|uç eder+ o| ||e ||ç||
|oo|. |eº|ouº +|e e\||ere|, p+|u|u|.


S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 159
FICük£ T-2T Fyoderma ¿ao¿reoosum A .e|, |+|çe u|ce| W||| |+|ºed |u||ouº uude|r|ued |o|de|º co.e|ed
W||| |ero|||+ç|c +ud |||||uouº e\ud+|e. w|eu ||e |u||+e +|e opeued, puº |º d|+|ued. I||º |eº|ou +|oºe +cu|e|,
+ud ºp|e+d |+p|d|, |o||oW|uç |+p+|+|or, |o| +u o.+||+u c+|c|uor+.
FICük£ T-28 Fyoderma ¿ao¿reoosum: chrooìc type I|e |eº|ou |u.o|.eº ||e uppe| e,e||d +ud |ep|eºeu|º
+u u|ce| W||| e|e.+|ed ç|+uu|+||uç |+ºe W||| ru|||p|e +|ºceººeº. I|e |eº|ou |+|e| ºp|e+d º|oW|, |o |u.o|.e ||e
|erpo|+| +ud /,çor+||c |eç|ouº +ud e.eu|u+||, |e+|ed uude| º,º|er|c ç|ucoco|||co|d ||e+|reu|, |e+.|uç + |||u
c||||||o|r ºc+| ||+| d|d uo| |rp+|| ||e |uuc||ou o| ||e e,e||d.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 160
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset Most 30-60 yeais.
Sex Women > men.
£tìo|o¿y Unknown, possibly hypeisensitivity
ieaction. Inflammatoiy bowel disease. SS is
counted among the neutiophilic deimatoses.
Assocìated 0ìsorders Febiile uppei iespiia-
toiy tiact infection. In some cases associated
with Yersínía infection. Hematologic malig-
nancy; diugs: gianulocyte colony-stimulating
factoi (G-GSF).
CIINICAI MANIF£SIAII0N
Piodiomes aie febiile uppei iespiiatoiy tiact
infections. Gastiointestinal symptoms (di-
aiihea), tonsillitis, influenza-like illness, 1-3
weeks befoie skin lesions. Lesions tendei/pain-
ful. Fevei (not always piesent), headache, ai-
thialgia, geneial malaise.
Skìo Iesìoos Biight ied, smooth, tendei
papules (2-4 mm in diametei) that coalesce to
foim iiiegulai, shaiply boideied, inflammatoiy
plaques (Fig. 7-30 À ). Pseudovesiculation:
intense edema gives the appeaiance of
vesiculation (Figs. 7-30 À and 7-31 À ). Lesions
Au uucorrou, +cu|e +ud |ecu||eu|, c,|o||ue·
|uduced º||u |e+c||ou +ººoc|+|ed W||| .+||ouº
e||o|oç|eº.
|+|u|u| p|+que·|o|r|uç |u||+rr+|o|, p+pu|eº,
o||eu W||| r+ºº|.e e\ud+||ouº ç|.|uç ||e +ppe+|·
+uce o| .eº|cu|+||ou (pºeudo.eº|cu|+||ou).
Aººoc|+|ed W||| |e.e|, +||||+|ç|+, +ud pe||p|e|+|
|eu|oc,|oº|º.
Aººoc|+|ed W||| |u|ec||ou, r+||çu+uc,, o| d|uçº.
I|e+|reu|. º,º|er|c ç|ucoco|||co|dº, po|+ºº|ur
|od|de, d+pºoue, o| co|c||c|ue.
',¤¤¤,¤. Acu|e |e||||e ueu||op||||c de|r+|oº|º.
SW££I S¥N0k0M£ (SS) |C|·9 . o95.39

|C|·¹0 . |93.2
FICük£ T-29 Fyoderma ¿ao¿reoosum: chrooìc type I||º |eº|ou, W||c| +ppe+|º |||e + p|+que, ºp|e+d
ou|, º|oW|, |u| W+º +|ºo ºu||ouuded |, +u e|,||er+|ouº |o|de|. I|e |eº|ou |º c|uº|ed +ud |,pe||e|+|o||c +ud |º
|eºº p+|u|u| ||+u ||e |eº|ouº |u +cu|e p,ode|r+ ç+uç|euoºur
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 161
FICük£ T-30 Sweet syodrome  Au e|,||er+|ouº, eder+|ouº p|+que ||+| |+º |o|red ||or co+|eºc|uç
p+pu|eº ou ||e ||ç|| c|ee|. I|e |o|de| o| ||e p|+que |oo|º +º || corpoºed o| .eº|c|eº, |u| p+|p+||ou |e.e+|º ||+|
|| |º ºo||d (pºeudo.eº|cu|+||ou). I||º |eº|ou occu||ed |u + 2o·,e+|·o|d |er+|e |o||oW|uç +u uppe| |eºp||+|o|, |u|ec·
||ou, +ud ||e p+||eu| +|ºo |+d |e.e| +ud |eu|oc,|oº|º.  A º|r||+| e|up||ou |u + 52·,e+|·o|d |er+|e. |o|e. ru|||p|e
co+|eºc|uç, |u||+rr+|o|, +ud .e|, e\ud+||.e p+pu|eº ou ||e uec|. I||º p+||eu| +|ºo |+d |eu|oc,|oº|º +ud |e.e|. |u
+dd|||ou, ||e|e |º .|||||ço.
 
 
FICük£ T-31 Sweet syodrome  Co+|eºc|uç e\ud+||.e p+pu|eº ||+| |oo| |||e .eº|c|eº. upou p+|p+||ou
|eº|ouº We|e ºo||d.  Bu||ouº |,pe o| 'Wee| º,ud|ore. I|eºe +|e ||ue |u||+e +ud puº|u|eº. I|e p+||eu| |+d
r,e|orouoc,||c |eu|er|+.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 162
aiise iapidly, and as they evolve, cential cleaiing
may lead to annulai oi aicuate patteins. Tiny,
supeificial pustules may occui. If associated
with leukemia, bullous lesions may occui (Fig.
7-31B ) and lesions may mimic pyodeima
gangienosum. May piesent as a single lesion
oi multiple lesions, asymmetiically distiibuted.
Most common on face (Fig. 7-30 À ), neck (Fig.
7-30B ), and uppei extiemities but also on lowei
extiemities, wheie Iesions may be deep in the
fat and thus mimic panniculitis oi eiythema
nodosum. Tiuncal lesions aie uncommon but
widespiead, and geneialized foims occui.
Mucous Membraoes ± Conjunctivitis, epis-
cleiitis.
Ceoera| £xamìoatìoo
Patient may appeai ill. Theie may be involve-
ment of caidiovasculai, cential neivous system,
gastiointestinal, hepatic, musculoskeletal, ocu-
lai, pulmonaiy, ienal, and splenic oigans.
IA80kAI0k¥ £XAMINAII0NS
Comp|ete 8|ood Couot Leukocytosis with neu-
tiophilia (not always peisistent).
£Sk Elevated.
0ermatopatho|o¿y Diagnostic. Epideimis
usually noimal but may show subcoineal pus-
tulation. Massive edema of papillaiy body,
dense leukocytic infiltiate with staibuist pat-
tein in mid-deimis, consisting of neutiophils
with occasional eosinophils/lymphoid cells.
Leukocytoclasia, nucleai dust, but othei signs
of vasculitis absent. ± Neutiophilic infiltiates in
subcutaneous tissue.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical impiession plus skin biopsy confiima-
tion.
very £dematous Acute F|aques Eiythema
multifoime, eiythema nodosum, pievesiculai
heipes simplex infection, pieulceiative pyo-
deima gangienosum.
C0ükS£ AN0 Fk0CN0SIS
Untieated, lesions enlaige ovei a peiiod of
days oi weeks and eventually iesolve without
scaiiing. With oial piednisone, lesions iesolve
within a few days. Recuiiences occui in 50%
of patients, often in pieviously involved sites.
Some cases follow Yersínía infection oi aie as-
sociated with acute myelocytic leukemia, tian-
sient myeloid piolifeiation, vaiious malignant
tumois, ulceiative colitis, benign monoclonal
gammopathy; some follow diug administia-
tion, most commonly by GSF.
MANAC£M£NI
Rule out sepsis.
Fredoìsooe 30-50 mg/d, tapeiing in 2-3
weeks; some, but not all, patients iespond to
dapsone, 100 mg/d, oi to potassium iodide.
Some to colchicine.
Aotìbìotìc Iherapy Cleais eiuption in Yersínía-
associated cases; in all othei cases antibiotics
aie ineffective.
S£CII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 163
A |+|e, |oc+||/ed |u||+rr+|o|, d|ºe+ºe o| uu|uoWu
e||o|oç,, c||u|c+||, c|+|+c|e||/ed |, |edd|º|·||oWu
p+pu|eº o| ºr+|| p|+queº p||r+|||, |u ||e |+ce.
'|uç|e o| ru|||p|e |eº|ouº W||| c|+|+c|e||º||c o|·
+uçe pee|·|||e ºu||+ce (||ç. 1·!2).
n|º|o|oç|c+||,, c||ou|c |eu|oc,|oc|+º||c .+ºcu||||º
W||| eoº|uop|||º, |||||u depoº|||ou, +ud ||||oº|º.
I|e|+p,. |op|c+| ç|ucoco|||co|dº, d+pºoue.

CkANüI0MA FACIAI£ (CF) |C|·9 . o3o.¹

|C|·¹0 . |92.2
FICük£ T-32 Craou|oma Iacìa|e: c|assìc preseotatìoo A º|uç|e, º|+|p|, de||ued, ||oWu p|+que W||| +
c|+|+c|e||º||c o|+uçe pee|·|||e ºu||+ce.
164
S E C I | 0 N 8
S£v£k£ AN0 IIF£-Ihk£AI£NINC
SkIN £küFII0NS IN Ih£
ACüI£I¥ III FAII£NI
EE' |º + ºe||ouº, +| ||reº |||e·|||e+|eu|uç, |e+c||ou
p+||e|u o| ||e º||u c|+|+c|e||/ed |, çeue|+||/ed
+ud uu||o|r |edueºº +ud ºc+||uç |u.o|.|uç p|+c||·
c+||, ||e eu|||e º||u.
|| |º +ººoc|+|ed W||| |e.e|, r+|+|ºe, º||.e|º, +ud
çeue|+||/ed |,rp|+deuop+||,, +ud |e.e|.
IWo º|+çeº, +cu|e +ud c||ou|c, re|çe oue |u|o ||e
o||e|. |u ||e +cu|e +ud ºu|+cu|e p|+ºeº, ||e|e |º
|+p|d ouºe| o| çeue|+||/ed .|.|d |ed e|,||er+
+ud ||ue ||+uu, ºc+|eº, ||e p+||eu| |ee|º |o| +ud
co|d, º||.e|º, +ud |+º |e.e|. |u c||ou|c EE', ||e
º||u |||c|euº, +ud ºc+||uç cou||uueº +ud |ecoreº
|+re||+|.
I|e|e |º + |oºº o| ºc+|p +ud |od, |+||, +ud ||e
u+||º |ecore |||c|eued +ud ºep+|+|ed ||or ||e
u+|| |ed (ou,c|o|,º|º).
I|e|e r+, |e |,pe|p|çreu|+||ou o| p+|c|, |oºº
o| p|çreu| |u p+||eu|º W|oºe uo|r+| º||u co|o| |º
||oWu o| ||+c|.
I|e roº| ||equeu| p|ee\|º||uç º||u d|ºo|de|º +|e
(|u o|de| o| ||equeuc,) pºo||+º|º, +|op|c de|r+·
||||º, +d.e|ºe cu|+ueouº d|uç |e+c||ou, |,rp|or+,
+||e|ç|c cou|+c| de|r+||||º, +ud p||,||+º|º |u||+
p||+||º.
|C|·9. o95.9
£XF0IIAIIv£ £k¥Ihk00£kMA S¥N0k0M£ (££S)
£FI0£MI0I0C¥
A¿e oI 0oset Usually >50 yeais; in childien,
EES usually iesults fiom pityiiasis iubia pilaiis
oi atopic deimatitis.
Sex Males > females.
£II0I0C¥
Some 50% of patients have histoiy of pieex-
isting deimatosis, which is iecognizable only
in the acute oi subacute stage. Most fiequent
pieexisting skin disoideis aie (in the oidei
of fiequency) psoiiasis, atopic deimatitis, ad-
veise cutaneous diug ieactions, cutaneous T
cell lymphoma, alleigic contact deimatitis, and
pityiiasis iubia pilaiis (Table 8-1). Diugs most
commonly implicated in EES aie shown in
Table 8-2. In 20% of patients it is not possible to
identify the cause by histoiy oi histology.
¦'ee ''e/+|, ',ud|ore¨ ('ec||ou 20) |o| + ºpec|+| couº|de|+||ou o| |||º |o|r o| EE'.|
IA8I£ 8-1 Etio|o¿y of E\fo|iative 0ermatitis
in Adu|ts
Cause
o
Avera¿e Ferceot
b
uude|e|r|ued o| uuc|+ºº|||ed 2!
|ºo||+º|º 2!
A|op|c de|r+||||º, ec/er+ ¹o
||uç +||e|ç, ¹5
|,rp|or+, |eu|er|+ ¹¹
A||e|ç|c cou|+c| de|r+||||º 5
'e|o|||e|c de|r+||||º 5
'|+º|º de|r+||||º W||| '|d¨ |e+c||ou !
|||,||+º|º |u||+ p||+||º 2
|erp||çuº |o||+ceuº ¹
c
|o| + corp|e|e ||º| o| d|ºe+ºeº +ººoc|+|ed W||| EE', ºee e\|o||+||.e
de|r+||||º p|c|u|e ç+||e|, |u ||e ou||ue .e|º|ou.
|
Aº co||+|ed ||or ||e |||e|+|u|e.
'ou|ce. A|||e.|+|ed ||or \n l|| e| +|, |u |\ ||eed|e|ç e| +| (edº).
|·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- o|| ed. |eW \o||,
\cC|+W·n|||, 200!.
S£CII0N 8 'E\EkE A|| |||E·InkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 165
FAIh0C£N£SIS
The metabolic iesponse to exfoliative deima-
titis may be piofound. Laige amounts of waim
blood aie piesent in the skin due to the dilata-
tion of capillaiies, and theie is consideiable
heat dissipation thiough insensible fluid loss
and by convection. Also, theie may be high-
output caidiac failuie; the loss of scales thiough
exfoliation can be consideiable, up to 9 g/m
2
of
body suiface pei day, and this may contiibute to
the ieduction in seium albumin and the edema
of the lowei extiemities so often noted in these
patients.
CIINICAI MANIF£SIAII0N
Depending on the etiology, the acute phase
may develop iapidly, as in a diug ieaction, lym-
phoma, eczema, oi psoiiasis. At this eaily acute
stage it is still possible to identify the pieexist-
ing deimatosis. Theie is fevei, piuiitus, fatigue,
weakness, anoiexia, weight loss, malaise, feeling
cold, shiveis.
Appearaoce oI Fatìeot Fiightened, ied, °toxic,"
may be malodoious.
Skìo Iesìoos Skin is ied, thickened, scaly.
Deimatitis is unifoim involving the entiie body
suiface (Figs. 8-1 to 8-3), except foi pityiiasis
IA8I£ 8-2 0ru¿s that Cause E\fo|iative 0ermatitis

A||opurìoo|
b
Code|ue \e|cu||+|º 'u||+º+|+/|ue
Ar|uoç|,coº|deº C,+u+r|de \eºu+ 'u||ou+r|deº
Ar|uop|,|||ue |+pºoue \e||,|p|edu|ºo|oue 'u||ou,|u|e+º
Ar|od+|oue ||deo\,|uoº|ue \|uoc,c||ue
Arou+||de ||||uu|º+| \||or,c|u C I+| p|ep+|+||ouº
Arp|c||||u ||p|eu,||,d+u|o|u 0rep|+/o|e Ie|||u+||ue
Au||r+|+||+|º Ep|ed||ue |eu|c||||u Ie||u|+||ue
A|ºeu|c+|º E||+r|u|o| |eu|oº|+||u I|+||dor|de
Aºp|||u E||,|eued|+r|ue |e||||+|e +ud I||+ce|+/oue
ç|,ce|,| |||u|||+|e
A/||eou+r E||e||u+|e ||eue|u||de I||+/|de d|u|e||cº
B+c|||r ||uo|ou|+c|| ||euo|p|||+|e|u I|c|op|d|ue
B+||||u|+|eº C\·C'| ||euo|||+/|ueº I|ro|o| r+|e+|e
B|orodeo\,u||d|ue Co|d ||eu,||u|+/oue e,ed|opº
Budeuoº|de ne||+| red|c+||ouº ||eu,|o|u Io||+r,c|u
Ca|cìum chaooe| |ude|o\+/|ue ||o|o||e|+p, Ioc+|u|de
b|ockers |,d|oc||o||de
C+p|op||| |ud|u+.|| ||+queu|| I||re||e\+|e
Carbamatepìoe |u|e||eu||u 2 ||+c|o|o| I|o.+||o\+c|u
C+||op|+||u |od|ue Çuìoìdìoe Iuro| uec|oº|º
Ce|o\|||u |ºou|+/|d k+u|||d|ue |+c|o|
Cep|+|oºpo||uº |ºoºo|||de d|u|||+|e ke||uo|dº \+ucor,c|u
Cìmetìdìoe |+uºop|+/o|e k||oº|+r,c|u \o||r||ue
C|ºp|+||u ||doc+|ue k||+rp|c|u /|do.ud|ue
C|od|ou+|e Iìthìum '|. lo|u'º Wo||
C|o|+/+r|ue \e||oqu|ue '||ep|or,c|u
c
|o| + ro|e e\|euº|.e ||º| o| d|uçº |rp||c+|ed |u EE', ºee e\|o||+||.e de|r+||||º º,ud|ore p|c|u|e ç+||e|, |u ||e ou||ue .e|º|ou.
|
I|e ro|e corrou|, |rp||c+|ed +çeu|º +|e ||º|ed |u |o|d.
'ou|ce. \n l||, A K|r,+|·Aº+d|, +ud |\ ||eed|e|ç, |u |\ ||eed|e|ç e| +|. (edº). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, o|| ed.
|eW \o||. \cC|+W·n|||, 200!. p.+31.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 166
iubia pilaiis, wheie EES spaies shaiply defined
aieas of noimal skin (see Fig. 5-7). Thickening
leads to exaggeiated skin folds (Figs. 8-2 and
8-3); scaling may be fine and bianny and may
be baiely peiceptible (Fig. 8-2) oi laige, up to
0.5 cm, and lamellai (Fig. 8-1).
Pu|ms und Sv|es Usually involved, with mas-
sive hypeikeiatosis and deep fissuies in pit-
yiiasis iubia pilaiis, Sézaiy syndiome, and
psoiiasis.
haìr Telogen effluvium, even alopecia, except
foi EES aiising in eczema oi psoiiasis.
Naì|s Thickening of nail plates, onycholysis,
shedding of nails.
Fì¿meotatìoo In chionic EES theie may be
hypeipigmentation oi patchy loss of pigment in
patients whose noimal skin is biown oi black.
Ceoera| £xamìoatìoo
Lymph nodes geneialized, iubbeiy, and usually
small; enlaiged in Sézaiy syndiome. Edema of
lowei legs and ankles.
IA80kAI0k¥ £XAMINAII0NS
Chemìstry Low seium albumin and inciease
in gammaglobulins; electiolyte imbalance;
acute-phase pioteins incieased.
hemato|o¿y Leukocytosis.
8acterìa| Cu|ture S|ín : iule out secondaiy
Sìa¡|y|otottus aureus infection. B|ooJ : iule out
sepsis.
0ermatopatho|o¿y Depends on type of un-
deilying disease. Paiakeiatosis, intei- and intia-
cellulai edema, acanthosis with elongation of
the iete iidges, and exocytosis of cells. Theie is
edema of the deimis and a chionic inflamma-
toiy infiltiate.
Ima¿ìo¿ CT scans oi MRI should be used to
find evidence of lymphoma.
Iymph Node 8ìopsy When theie is suspicion
of lymphoma.
0IACN0SIS
Diagnosis is not easy, and the histoiy of the
pieexisting deimatosis may be the only clue.
Also, pathognomonic signs and symptoms
of the pieexisting deimatosis may help, e.g.,
dusky-ied coloi in psoiiasis and yellowish-ied
in pityiiasis iubia pilaiis; typical nail changes
of psoiiasis; lichenification, eiosions, and ex-
coiiations in atopic deimatitis and eczema;
diffuse, ielatively nonscaling palmai hypei-
keiatoses with fissuies in cutaneous T cell
lymphoma (CTCL) and pityiiasis iubia pilaiis;
shaiply demaicated patches of noninvolved
skin within the eiythiodeima in pityiiasis
iubia pilaiis; massive hypeikeiatotic scale of
scalp, usually without haii loss in psoiiasis and
with haii loss in CTCL and pityiiasis iubia
pilaiis; in the lattei and in CTCL, ectiopion
may occui.
C0ükS£ AN0 Fk0CN0SIS
Guaided, depends on undeilying etiology. De-
spite the best attention to all details, patients
may succumb to infections oi, if they have cai-
diac pioblems, to caidiac failuie (°high-output"
failuie) oi, as was often the case in the past, to
the effects of piolonged glucocoiticoid theiapy.
MANAC£M£NI
This is an impoitant medical pioblem that
should be dealt with in a modein inpatient dei-
matology facility with expeiienced peisonnel.
The patient should be hospitalized in a single
ioom, at least foi the beginning woikup and
duiing the development of a theiapeutic pio-
giam. The hospital ioom conditions (heat and
cold) should be adjusted to the patient's needs;
most often these patients need a waim ioom
with many blankets.
Iopìca| Watei baths with added bath oils, fol-
lowed by application of bland emollients.
Systemìc Oial glucocoiticoids foi iemission
induction but not foi maintenance; sysìemít
anJ ìo¡íta| ì|era¡y as requíreJ |y unJer|yíng
tonJíìíon.
Supportìve Suppoitive caidiac, fluid, electio-
lyte, piotein ieplacement theiapy as iequiied.
S£CII0N 8 'E\EkE A|| |||E·InkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 16T
FICük£ 8-1 £xIo|ìatìve dermatìtìs: psorìasìs I|e|e |º uu|.e|º+| e|,||er+, |||c|eu|uç o| ||e º||u, +ud
|e+., ºc+||uç. I||º p+||eu| |+d pºo||+º|º +º ºuççeº|ed |, ||e |+|çe º||.e|, W|||e ºc+|eº +ud ||e ºc+|p +ud u+||
|u.o|.ereu| uo| ºeeu |u |||º |||uº||+||ou. I|e p+||eu| |+d |+||çue, We+|ueºº, r+|+|ºe, +ud W+º º||.e||uç. || |º qu||e
o|.|ouº ||+| ºuc| r+ºº|.e ºc+||uç c+u |e+d |o p|o|e|u |oºº +ud ||e r+\|r+| d||+|+||ou o| º||u c+p|||+||eº |o couº|d·
e|+||e |e+| d|ºº|p+||ou +ud ||ç|·ou|pu| c+|d|+c |+||u|e
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 168
FICük£ 8-2 £xIo|ìatìve dermatìtìs: dru¿-ìoduced I||º |º çeue|+||/ed e|,|||ode|r+ W||| |||c|eu|uç o|
º||u |eºu|||uç |u |uc|e+ºed º||u |o|dº, uu|.e|º+| |edueºº, + ||ue ||+Wu, ºc+||uç. I||º p+||eu| |+d de.e|oped e|,|||o·
de|r+ |o||oW|uç ||e |ujec||ou o| ço|d º+||º |o| ||eur+|o|d +|||||||º.
S£CII0N 8 'E\EkE A|| |||E·InkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 169
FICük£ 8-3 £xIo|ìatìve dermatìtìs: cutaoeous I ce|| |ymphoma I|e|e |º uu|.e|º+| e|,||er+, |||c|eu|uç,
+ud ºc+||uç. |o|e ||+| |u cou||+º| |o e|,|||ode|r+ º|oWu |u ||çº. 3·¹ +ud 3·2 ||e deç|ee o| e|,||er+ +ud |||c|·
ueºº |º uo| uu||o|r +ud ||e |edueºº |+º + ||oWu|º| |ue. |u +dd|||ou, |||º e|de||, p+||eu| |+d |+|| |oºº, r+ºº|.e
|u.o|.ereu| o| p+|rº +ud ºo|eº W||| d|||uºe |,pe||e|+|oºeº, c|+c|º, +ud ||ººu|eº. Ceue|+||/ed |,rp|+deuop+||,
W+º +|ºo p|eºeu|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 1T0
I|e ºuddeu +ppe+|+uce o| + |+º| +ud |e.e| c+uºe
+u\|e|, |o| ||e p+||eu|. \ed|c+| +d.|ce |º ºouç||
|rred|+|e|, +ud o||eu |u ||e ere|çeuc, uu||º
o| |oºp||+|º, +|ou| ¹0° o| +|| p+||eu|º ºee||uç
ere|çeuc, red|c+| c+|e |+.e + de|r+|o|oç|c
p|o||er.
I|e d|+çuoº|º o| +u +cu|e |+º| W||| + |e.e| |º +
c||u|c+| c|+||euçe (||çº. 3·+ +ud 3·5) || + d|+çuoº|º
|º uo| eº|+|||º|ed p|orp||, |u ce||+|u p+||eu|º
¦e.ç., ||oºe |+.|uç ºep||cer|+ (||ç. 3·o)|, |||eº+.|uç
||e+|reu| r+, |e de|+,ed.
I|e cu|+ueouº ||ud|uçº +|oue +|e o||eu d|+çuoº||c
|e|o|e cou|||r+|o|, |+|o|+|o|, d+|+ +|e +.+||+||e.
Aº |u p|o||erº o| ||e +cu|e +|doreu, ||e |eºu||º
o| ºore |+|o|+|o|, |eº|º, ºuc| +º r|c|o||o|oç|c
cu||u|eº, r+, uo| |e +.+||+||e |rred|+|e|,. 0u
||e |+º|º o| + d|||e|eu||+| d|+çuoº|º, +pp|op||+|e
||e|+p,-W|e||e| +u||||o||cº o| ç|ucoco|||co|dº-
r+, |e º|+||ed. |u|||e|ro|e, p|orp| d|+çuoº|º
+ud |ºo|+||ou o| ||e p+||eu| W||| + cou|+ç|ouº
d|ºe+ºe, W||c| r+, |+.e ºe||ouº couºequeuceº,
p|e.eu| ºp|e+d |o o||e| pe|ºouº. |o| e\+rp|e,
.+||ce||+ |u +du||º (ºee ||çº. 21·!9 +ud 21·+0)
|+|e|, c+u |e |+|+|. Cou|+ç|ouº d|ºe+ºeº p|eºeu|·
|uç W||| |+º| +ud |e.e| +º ||e r+jo| ||ud|uçº
|uc|ude .··c| ·¤|-:|·¤¤º (||ç. 3·5), eu|e|o.||uº, +ud
p+|.o.||uº |u|ec||ouº (ºee ||ç. 21·2+ 1 3) +ud
|c:|-··c| ·¤|-:|·¤¤º ¦º||ep|ococc+| (ºee ||ç. 2+·+3),
º|+p|,|ococc+| (ºee ||ç. 2+·+0), reu|uçococc+|
(ºee ||çº. 2+·50, 2+·5¹), |,p|o|d, +ud º,p||||º (ºee
||ç. !0·2¹)|.
I|e p|,º|c+| d|+çuoº|º o| º||u e|up||ouº |º + d|ºc|·
p||ue |+ºed r+|u|, ou p|ec|ºe |deu||||c+||ou o| ||e
|,pe o| º||u |eº|ou. I|e p|,º|c|+u ruº| uo| ou|,
|deu|||, +ud c|+ºº||, ||e |,¤- o| º||u |eº|ou |u|
+|ºo |oo| |o| +dd|||ou+| ro|p|o|oç|c c|ueº ºuc| +º
||e :¤¤|·¸±·c|·¤¤ (+uuu|+|! |||º!) o| ||e |ud|.|du+|
|eº|ou, ||e c··c¤¸-¤-¤| (/oº|e|||o|r! ||ue+|!)
o| ||e |eº|ouº, ||e !·º|··|±|·¤¤ ¤c||-·¤ (e\poºed
+|e+º! ceu|||pe|+| o| ceu||||uç+|! rucouº rer·
||+ueº!).
 |u ||e !·||-·-¤|·c| !·c¸¤¤º·º o| e\+u||erº || |º
|rpo||+u| |o de|e|r|ue, |, ||º|o|,, ||e º·|- ¤|
|··º| c¤¤-c·c¤:- ¦||e |+º| o| koc|, \ouu|+|u
ºpo||ed |e.e| c|+|+c|e||º||c+||, +ppe+|º |||º| ou ||e
W||º|º +ud +u||eº (ºee ||çº. 2o·¹, 2o·2)|, +ud .e|,
|rpo||+u| |º ||e |-¤¤¤·c| -.¤|±|·¤¤ o| ||e |+º|.
¦\e+º|eº (ºee ||çº. 3·5 +ud 21·22) ºp|e+dº ||or
|e+d |o |oeº |u + pe||od o| ! d+,º, W|||e ||e |+º|
o| |u|e||+ (ºee ||ç. 21·2¹) ºp|e+dº |+p|d|, |u 2+
|o +3 | ||or |e+d |o |oeº +ud ||eu ºequeu||+||,
c|e+|º-|||º| |+ce, ||eu ||uu|, +ud ||eu ||r|º.|
A|||ouç| ||e|e r+, |e ºore o.e||+p, ||e d||·
|e|eu||+| d|+çuoº||c poºº|||||||eº r+, |e ç|ouped
|u|o ||.e r+|u c+|eço||eº +cco|d|uç |o ||e |,pe o|
|eº|ou (I+||e 3·!).
kASh£S IN Ih£ ACüI£I¥ III F£8kII£ FAII£NI
IA80kAI0k¥ I£SIS AvAIIA8I£ F0k ÇüICk
0IACN0SIS
The physician should make use of the following
laboiatoiy tests immediately oi within 8 h:
1 Díretì smear [rom ì|e |ase o[ a ·esít|e. This
pioceduie, known as the T:ant| ìesì , is de-
sciibed in the Intioduction. Smeais aie
examined foi acantholytic cells, giant acan-
thocytes, and/oi multinucleated giant cells
(see Fig. 27-27).
2 Víra| tu|ìure , negative stain (election micio-
scopy), polymeiase chain ieaction foi infec-
tions with heipes viiuses, diiect fluoiescence
(DIF) technique.
3 Cram sìaín o[ as¡íraìes or stra¡íng . This is es-
sential foi piopei diagnosis of pustules. Oi-
ganisms can be seen in the lesions of acute
meningococcemia, iaiely in the skin lesions
of gonococcemia and ecthyma gangienosum.
4 Tout| ¡re¡araìíon . This is especially helpful
in deep fungal infections and leishmaniasis.
The deimal pait of a skin biopsy specimen is
touched iepeatedly to a glass slide; the touch
piepaiation is ímmeJíaìe|y fixed in 95% ethyl
alcohol. Special stains aie then peifoimed,
and the slide examined foi oiganisms in the
cytology laboiatoiy.
5 Bío¡sy o[ ì|e s|ín |esíon. All puipuiic lesions
should be biopsied. Inflammatoiy deimal
nodules and most ulceis should be biopsied
(at base and maigin) and a poition of tissue
minced and cultuied foi bacteiia and fungi. A
3- to 4-mm tiephine and local anesthesia aie
used. In many laboiatoiies the biopsy speci-
men can be piocessed within 8 h if necessaiy.
In gangienous cellulitis (see Section 24) fio-
zen sections of a deep biopsy will veiify the
diagnosis in minutes.
S£CII0N 8 'E\EkE A|| |||E·InkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 1T1
FICük£ 8-4 Ceoera|ìted Iìxed dru¿ eruptìoo: tetracyc|ìoe. ||oº||+|ed, 59·,e+|·o|d Wor+u W||| |e.e|.
\u|||p|e cou||ueu| .|o|+ceouº |ed e|,||er+|ouº +|e+º, ºore o| W||c| |+|e| |ec+re |u||ouº.
FICük£ 8-5 Ceoera|ìted rash wìth Iever: meas|es \ouuç Wor+u W||| ||ç| |e.e|, couç|, coujuuc||·
.|||º, +ud + cou||ueu| r+cu|op+pu|+| e|up||ou |u ||e eder+|ouº |+ce. I|e |+º| +|ºo |u.o|.eº ||e ||uu| +ud ||e
e\||er|||eº. I|e p+||eu| |+º re+º|eº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 1T2
6 B|ooJ anJ uríne examínaìíons . Blood cultuie,
iapid seiologic tests foi syphilis, and seiology
foi lupus eiythematosus iequiie 24 h. Exami-
nation of uiine sediment may ieveal ied cell
casts in alleigic vasculitis.
7 Dar|-[íe|J examínaìíon . In the skin lesions
of secondaiy syphilis, iepeated examination
IA8I£ 8-3 Cenera|ited Eruptions in the Acute|y ||| Patient: 0ia¿nosis Accordin¿ to Iype of Lesion
c
Ceoera|ìted Ceoera|ìted 0ìseases MaoìIested
Ceoera|ìted £ruptìoos £ruptìoos MaoìIested by Wìdespread
Ceoera|ìted £ruptìoos £ruptìoos MaoìIested by by Furpurìc Macu|es, £rythema  Fapu|es
MaoìIested by MaoìIested by vesìc|es, 8u||ae, Furpurìc Fapu|es, Fo||owed by
Macu|es, Fapu|es Whea|s, F|aques or Fustu|es or Furpurìc vesìc|es 0esquamatìoo
||uç |,pe|ºeuº|||.|||eº 'e|ur º|c|ueºº ||uç ||uç |,pe|ºeuº|||.|||eº ||uç |,pe|ºeuº|||.|||eº
Acu|e n|\ º,ud|ore 'Wee| º,ud|ore |,pe|ºeuº|||.|||eº \eu|uçococcer|+
|
'|+p|,|ococc+|
E|,||er+ |u|ec||oºur Acu|e u|||c+||+ A||e|ç|c cou|+c| (+cu|e o| c||ou|c) ºc+|ded·º||u
(p+|.o.||uº B¹9) E|,||er+ de|r+||||º ||or Couococcer|+
|
º,ud|ore
C,|oreç+|o.||uº, r+|ç|u+|ur p|+u|º '|+p|,|ococcer|+ Io\|c º|oc| º,ud|ore
p||r+|, |u|ec||ou k|c|e||º|+|po\ |º-±!¤¤¤¤cº K+W+º+|| º,ud|ore
Epº|e|u·B+|| .||uº, Couococcer|+ |+c|e|er|+ C|+||·.e|ºuº·|oº|
p||r+|, |u|ec||ou \+||ce||+ 'u|+cu|e |+c|e||+| |e+c||ou
E\+u||er ºu|||ur (c||c|eupo\)
:
eudoc+|d|||º E|,|||ode|r+
(nn\ o) Ec/er+ |e|pe||cur
:
Eu|e|o.||uº |u|ec||ouº (e\|o||+||.e
Eu|e|o.||uº |u|ec||ouº (ec|o.||uº, Co\º+c||e) de|r+||||º)
\e+º|eº (|u|eo|+) (Co\º+c||e), k|c|e||º|+| d|ºe+ºeº.
Ce|r+u re+º|eº (|u|e||+)
!
|uc|ud|uç |+ud·|oo|· koc|, \ouu|+|u
Eu|e|o.||uº |u|ec||ouº +ud·rou|| d|ºe+ºe ºpo||ed |e.e|
(ec|o.||uº +ud Co\º+c||e) Io\|c ep|de|r+| I,p|uº, |ouºe·|o|ue
Adeuo.||uº |u|ec||ouº uec|o|,º|º (ep|der|c)
'c+||e| |e.e| 'r+||po\ o| .+||o|+ 'A||e|ç|c¨ .+ºcu||||º
|
E||||c||oº|º '|+p|,|ococc+| ||ººer|u+|ed
I,p|o|d |e.e| ºc+|ded·º||u |u||+.+ºcu|+|
'ecoud+|, º,p||||º º,ud|ore co+çu|+||ou (pu|pu|+
I,p|uº, ru||ue (euder|c) E|,||er+ ru||||o|re |u|r|u+uº
|
)
koc|, \ouu|+|u ºpo||ed K+W+º+|| d|ºe+ºe l·|··¤ |u|ec||ouº
|e.e| (e+||, |eº|ouº)
!
.ou /ur|uºc|
0||e| ºpo||ed |e.e|º puº|u|+| pºo||+º|º
||ººer|u+|ed deep |uuç+| Acu|e ç|+||·.e|ºuº·
|u|ec||ou |u |rruuo· |oº| |e+c||ou
corp|or|ºed p+||eu|º
E|,||er+ ru||||o|re
',º|er|c |upuº e|,||er+|oºuº
Acu|e ç|+||·.e|ºuº·|oº| |e+c||ou
of papules may show Tre¡onema ¡a||íJum .
The daik-field examination is not ieli-
able in the mouth because nonpathogenic
oiganisms aie almost impossible to dif-
feientiate fiom T. ¡a||íJum , but a lymph
node aspiiate can be subjected to daik-field
examination.
c
w||| |eç+|d |o ||e de|+||ed ro|p|o|oç|eº, ||e |e+de| |º
|e|e||ed |o ||e
|eºpec||.e ºec||ouº.
|
0||eu p|eºeu| +º |u|+|c|º.
:
ur||||c+|ed .eº|c|eº.
!
\+, |+.e +||||+|ç|+ o| ruºcu|oº|e|e|+| p+|u.
-
|e+d|uç |o |+|çe +|e+º o| ||+c| uec|oº|º.
S£CII0N 8 'E\EkE A|| |||E·InkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 1T3
0£FINIII0N
TEN is a maximal vaiiant of SJS diffeiing only
in the extent of body suiface involvement.
Both can stait with maculai and taiget-like like
lesions; howevei, about 50% of TEN cases do
'l' +ud IE| +|e +cu|e |||e·|||e+|eu|uç ruco·
cu|+ueouº |e+c||ou c|+|+c|e||/ed |, e\|euº|.e
uec|oº|º +ud de|+c|reu| o| ||e ep|de|r|º.
I|e, +|e ºe.e|e .+||+u|º o| +u |deu||c+| p+||o|oç|c
p|oceºº +ud d|||e| ou|, |u ||e pe|ceu|+çe o| |od,
ºu||+ce |u.o|.ed.
E|||e| |d|op+|||c o| d|uç·|uduced.
|+||orec|+u|ºr |º W|deºp|e+d +pop|oº|º o| |e·
|+||uoc,|eº |uduced |, + ce||·red|+|ed c,|o|o\|c
|e+c||ou.
Cou||ueu| e|,||er+|ouº pu|pu||c +ud |+|çe|·|||e
r+cu|eº e.o|.e |u|o ||+cc|d |||º|e|º +ud ep|de|r+|
de|+c|reu| roº||, ou ||e ||uu| +ud e\||er|·
||eº, +ud ||e|e |º +ººoc|+|ed rucouº rer||+ue
|u.o|.ereu|.
n|º|op+||o|oç|c+||,. |u||·|||c|ueºº uec|oº|º o| ||e
ep|de|r|º +ud + ºp+|ºe |,rp|oc,||c |u|||||+|e.
I|e+|reu| |º º,rp|or+||c. ',º|er|c ||e+|reu|
W||| ç|ucoco|||co|dº +ud ||ç|·doºe |u||+.euouº
|rruuoç|o|u||u |º cou||o.e|º|+|.
SI£v£NS-l0hNS0N S¥N0k0M£ (SlS) AN0 I0XIC £FI0£kMAI
N£Ck0I¥SIS (I£N) |C|·9 . o95.¹

|C|·¹0 . |5¹.¹/5¹.2
FICük£ 8-6 Ceoera|ìted purpura oecrosìs aod Iever: 0IC 5+·,e+|·o|d Wor+u W||| |e.e|, p|oº||+||ou,
+ud e\|euº|.e çeoç|+p||c |u|+|c||ouº ou ||e |+ce, ||e ||uu|, +ud ||e e\||er|||eº. I||º |º d|ººer|u+|ed |u||+.+ºcu|+|
co+çu|+||ou. pu|pu|+ |u|r|u+uº |o||oW|uç ºepº|º +||e| +|dor|u+| ºu|çe|,.
not, and in these the condition evolves fiom
diffuse eiythema to immediate neciosis and
epideimal detachment.
Theie is now consensus that SJS and TEN aie
diffeient fiom eiythema multifoime(EM).
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 1T4
SJS. <10% epideimal detachment
SJS/ TEN o·er|a¡. 10-30% epideimal detach-
ment.
TEN. >30% epideimal detachment.
£FI0£MI0I0C¥
A¿e oI 0oset Any age, but most common in
adults >40 yeais. Equal sex incidence.
0vera|| Iocìdeoce TEN : 0.4-1.2 pei million
peison-yeais. SJS : 1.2-6 pei million peison-
yeais.
kìsk Factors Systemic lupus eiythematosus,
HLA-B12, HIV/AIDS.
£II0I0C¥ AN0 FAIh0C£N£SIS
Polyetiologic ieaction pattein, but diugs aie
cleaily the leading causative factoi. TEN : 80%
of cases have stiong association with specific
medication (Table 8-4); <5% of patients ie-
poit no diug use. Also: chemicals, Myto¡|asma
pneumoniae, viial infections, immunization.
SJS : 50% aie associated with diug exposuie;
etiology often not cleai-cut.
Pathogenesis of SJS-TEN is only paitially
undeistood. It is viewed as a cytotoxic im-
mune ieaction aimed at the destiuction of
keiatinocytes expiessing foieign (diug-ie-
lated) antigens. Epideimal injuiy is based
on the induction of apoptosis. Diug-specific
activation of T cells has been shown in vitio
on peiipheial blood mononucleai cells of
patients with diug eiuptions. The natuie of
the antigens that diive the cytotoxic cellulai
immune ieaction is not well undeistood.
Diugs oi theii metabolites act as haptens and
iendei keiatinocytes antigenic by binding to
theii suifaces. Cutaneous diug eiuptions have
been linked to a defect of the detoxification
systems of livei and skin, which iesults in
diiect toxicity oi alteiation of antigenic piop-
eities of keiatinocytes. Cytokines pioduced by
activated mononucleai cells and keiatinocytes
piobably contiibute to local cell death, fevei,
and malaise.
CIINICAI MANIF£SIAII0N
Time fiom fiist diug exposuie to onset of
symptoms: 1-3 weeks. Occuis moie iapidly
with iechallenge. Often aftei days of ingestion
of the diug; newly added diug is most sus-
pect. Piodiomes: fevei, malaise, aithialgias 1-3
days piioi to mucocutaneous lesions. Mild to
modeiate skin tendeiness, conjunctival buining
IA8I£ 8-4 Nedications and the Risk of Io\ic Epiderma| Necro|ysis
h|gh 8|sk Lower 8|sk 0o0btI0| 8|sk ho £v|deoce oI 8|sk
A||opu||uo| Ace||c +c|d |'A||º |+|+ce|+ro| Aºp|||u
(e.ç., d|c|o|eu+c) (+ce|+r|uop|eu)
'u||+re||o\+/o|e Ar|uopeu|c||||uº |,|+/o|oue +u+|çeº|cº 'u||ou,|u|e+
'u||+d|+/|ue Cep|+|oºpo||uº Co|||coº|e|o|dº I||+/|de d|u|e||cº
'u||+p,||d|ue 0u|uo|oueº 0||e| |'A||º (e\cep| +ºp|||u) |u|oºer|de
'u||+do\|ue C,c||uº 'e|||+||ue A|d+c|oue
'u||+º+|+/|ue \+c|o||deº C+|c|ur c|+uue| ||oc|e|º
C+||+r+/ep|ue B|oc|e|º
|+ro|||ç|ue Auç|o|euº|u·cou.e|||uç eu/,re |u|||||o|º
||euo|+||||+| Auç|o|euº|u || |ecep|o| +u|+çou|º|º
||eu,|o|u '|+||uº
||eu,||u|+/oue no|roueº
|e.||+p|ue \||+r|uº
0\|c+r |'A||º
I||+ce|+/oue
|'A||º, uouº|e|o|d+| +u||·|u||+rr+|o|, d|uçº.
'ou|ce. |. \+|e,||e·A||+uo|e, l·C kouje+u. Ep|de|r+| uec|o|,º|º, |u |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, 1e, K wo||| e| +| (edº).
|eW \o|| , \cC|+W·n|||, 2003, C|+p. !9.
S£CII0N 8 'E\EkE A|| |||E·InkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 1T5
oi itching, then skin pain, buining sensation,
tendeiness, paiesthesia. Mouth lesions aie pain-
ful, tendei. Impaiied alimentation, photopho-
bia, painful mictuiition, anxiety.
Skìo Iesìoos Prvdrvmu| Rush Is moibillifoim,
can be taiget lesion-like, with/without puipuia
(Fig. 8-7); iapid confluence of individual lesions
(Fig. 8-8); alteinatively, can stait with diffuse
eiythema (Fig. 8-9).
Eur|y Neciotic epideimis fiist appeais as
maculai aieas with ciinkled suiface that enlaige
and coalesce (Fig. 8-7). Sheetlike loss of epidei-
mis (Figs. 8-7 to 8-9). Raised flaccid blisteis that
spiead with lateial piessuie (Nikolsky sign) on
eiythematous aieas. With tiauma, full-thickness
epideimal detachment yields exposed, ied, ooz-
ing deimis (Figs.8-8 and 8-9) iesembling a
second-degiee theimal buin.
Recvvery Regiowth of epideimis begins
within days; completed in >3 weeks. Piessuie
points and peiioiificial sites exhibit delayed
healing. Skin that is not denuded acutely is
shed in sheets, especially palms/soles. Nails and
eyelashes may be shed.
DIstrIhutIvn Initial eiythema on face, ex-
tiemities, becoming confluent ovei a few houis
oi days. Epideimal sloughing may be geneial-
ized, iesulting in laige denuded aieas (Figs. 8-8
and 8-9). Scalp, palms, soles may be less seveiely
involved oi spaied. SJS : widely distiibuted with
piominent involvement of tiunk and face. TEN :
geneialized, univeisal.
Mucous Membraoes Invaiiably involved, i.e.,
eiythema, painful eiosions: lips, buccal mucosa,
conjunctiva, genital and anal skin.
£yes 85% have conjunctival lesions: hypei-
emia, pseudomembiane foimation; keiatitis,
coineal eiosions; latei synechiae between eye-
lids and bulbai conjunctiva.
haìr aod Naì|s Eyelashes and nails may be
shed in TEN.
C£N£kAI FIN0INCS
· Fevei usually highei in TEN than in SJS.
· Usually mentally aleit. Distiess due to seveie
pain.
· Caidiovasculai : pulse may be >120 beats/
min. Blood piessuie.
· Renal: tubulai neciosis may occui. Acute
ienal failuie.
· Respiiatoiy and GI tiacts: sloughing of epi-
thelium with eiosions.
FICük£ 8-T I£N, exaothematìc preseotatìoo I|e|e |º + W|deºp|e+d cou||ueu| r+cu|+| |+º| W||| c||u|||uç
o| ||e ep|de|r|º |u ºore +|e+º +ud de|+c|reu| o| ||e ep|de|r|º |u o||e|º, |e+.|uç oo/|uç |ed e|oº|ouº. I||º
e|up||ou W+º due |o +||opu||uo|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 1T6
IA80kAI0k¥ £XAMINAII0NS
hemato|o¿y Anemia, lymphopenia; eosi-
nophilia uncommon. Neutiopenia coiielates
with pooi piognosis. Seium uiea incieased,
seium bicaibonate decieased.
0ermatopatho|o¿y Eur|y Vacuolization/
neciosis of basal keiatinocytes and individual
cell neciosis (apoptosis) thioughout the epi-
deimis.
Lute Full-thickness epideimal neciosis and
detachment with foimation of subepideimal
split above basement membiane. Spaise lym-
phocytic infiltiate in deimis. Immunofluoies-
cence studies uniemaikable, iuling out othei
blisteiing disoideis.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
£ar|y Exanthematous diug eiuptions, EM
majoi, scailet fevei, phototoxic eiuptions, toxic
shock syndiome, giaft-veisus-host disease
(GVHD).
Fu||y £vo|ved EM majoi (typical taiget le-
sions, piedominantly on extiemities), GVHD
(may mimic TEN; less mucosal involvement),
theimal buins, phototoxic ieactions, staphylo-
coccal scalded-skin syndiome (in young chil-
dien, iaie in adults), geneialized bullous fixed
diug eiuption, exfoliative deimatitis.
C0ükS£ AN0 Fk0CN0SIS
Aveiage duiation of piogiession is <4 days. A
piognostic scoiing system is shown in Table
8-5. Couise similai to that of extensive theimal
buins. Piognosis ielated to extent of skin necio-
sis. Tianscutaneous fluid loss is laige and vaiies
with aiea of denudation; associated electio-
lyte abnoimalities. Pieienal azotemia common.
Bacteiial colonization common and associated
with sepsis. Othei complications include hy-
peimetabolic state and diffuse inteistitial pneu-
monitis. Moitality iate foi TEN is 30%, mainly
in eldeily; foi SJS, 5-12%. Moitality ielated to
sepsis, GI hemoiihage, fluid/electiolyte imbal-
ance. If the patient suivives the fiist episode of
SJS/TEN, ieexposuie to the causative diug may
be followed by iecuiience within houis to days,
moie seveie than the initial episode.
S£Çü£IA£
S|In Scaiiing, hypo- and hypeipigmentation,
eiuptive nevomelanocytic nevi, abnoimal ie-
giowth of nails.
Eyes Common, including Sjögien-like sicca
syndiome with deficiency of mucin in teais;
entiopion, tiichiasis, squamous metaplasia,
neovasculaiization of conjunctiva and coinea;
synblephaion, punctate keiatitis, coineal scai-
iing; peisistent photophobia, buining eyes,
visual impaiiment, blindness.
AnvgenItu|Iu: Phimosis, vaginal synechiae.
MANAC£M£NI
Acute SJS/TEN
· Eaily diagnosis and withdiawal of suspected
diug(s) aie veiy impoitant.
· Patients aie best caied foi in an inteimediate
oi intensive caie unit.
IA8I£ 8-5 SC0RIEN: A Pro¿nostic
Scorin¿ System for Patients with Epiderma|
Necro|ysis

SC0kI£N
Fro¿oostìc Factors
Foìots
 Açe > +0 ,|
 ne+|| |+|e > ¹20 |e+|º/r|u
 C+uce| o| |er+|o|oç|c
r+||çu+uc,
 Bod, ºu||+ce +|e+ |u.o|.ed
> ¹0 pe|ceu|
 'e|ur u|e+ |e.e| > ¹0 r!
 'e|ur ||c+||ou+|e |e.e|
· 20 r!
 'e|ur ç|ucoºe |e.e| > ¹+ r!
SC0kI£N
0-¹
2
!
+
> 5
¹
¹
¹
¹
¹
¹
¹
Morta|ìty
kate (%)
!.2
¹2.¹
!5.3
53.!
90
c
|¤|- |, c±||¤·º. A|||ouç| || |º ||ç||, +pp|ec|+|ed ||+| We uoW
|+.e + ºco||uç º,º|er, We do |+.e + |eºe|.+||ou W||| 'C0kIE|.
0u|, oue po|u| |º +ºº|çued |o |od, ºu||+ce +|e+ |u.o|.ereu|
(>¹0°). I|e|e |º de||u||e|, + p|oçuoº||c d|||e|euce |e|Weeu, e.ç.,
20° +ud 10° |od, ºu||+ce +|e+ |u.o|.ereu| +ud |||º º|ou|d |e
|e||ec|ed |u ||e |o|+| ºco|e.
'ou|ce. |+|+ ||or ' B+º|uj|·C+||u e| +|. 'C0kIE|. A ºe.e|||,·o|·
|||ueºº ºco|e |o| |o\|c ep|de|r+| uec|o|,º|º. / |¤.-º| |-·¤c|¤| 115.
¹+9, 2000, ||or | \+|e,||e·A||+uo|e, l·C kouje+u. Ep|de|r+| uec|o|·
,º|º, |u |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, 1|| ed,
K wo||| e| +| (edº). |eW \o|| , \cC|+W·n|||, 2003, C|+p. !9.
S£CII0N 8 'E\EkE A|| |||E·InkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 1TT
· Manage ieplacement of IV fluids and elec-
tiolytes as foi patient with a thiid-degiee
theimal buin. Howevei, less fluid usually
iequiied as foi theimal buin of similai
extent.
· Systemic glucocoiticoids eaily in the disease
and in high doses aie iepoited helpful in
ieducing moibidity oi moitality (as is also
the expeiience of the authois), but this has
been questioned. Late in the disease they aie
contiaindicated.
· High-dose IV immunoglobulins halt piogies-
sion of TEN if administeied eaily. This is
questioned by some authois; the disciepancy
may be explained by the diffeient pioducts
and batches used.
· Pentoxifylline IV by continuous diip eaily on
in the eiuption has been anecdotally iepoited
to be beneficial.
· With oiophaiyngeal involvement, suction fie-
quently to pievent aspiiation pneumonitis.
· Suigical debiidement not iecommended.
· Diagnose and tieat complicating infections,
including sepsis (fevei, hypotension, change
in mental status).
· Tieat eye lesions eaily with eiythiomycin
ointment.
Freveotìoo The patient must be awaie of the
likely offending diug and that othei diugs of
the same class can cioss-ieact. These diugs
must nevei be ieadministeied. Patient should
weai a medical aleit biacelet.
FICük£ 8-8 I£N, exaothematìc preseotatìoo
A r+cu|+| |+º| ||+| |º º|||| .|º|||e ou ||e |oWe| ||+u|
|+º co+|eºced, +ud d|º|odçreu| +ud º|edd|uç o| ||e
uec|o||c ep|de|r|º |+º |ed |o |+|çe, oo/|uç, e\||ere|,
p+|u|u| e|oº|ouº. I||º |eºer||eº ºc+|d|uç. I|e e|up||ou
W+º due |o + ºu||ou+r|de.
FICük£ 8-9 I£N, ooo-exaothematìc dìIIuse
preseotatìoo I||º o0·,e+|·o|d r+u de.e|oped
d|||uºe e|,||er+ o.e| +|roº| ||e eu|||e |od,,
W||c| ||eu |eºu||ed |u ep|de|r+| c||u|||uç, de|+c|·
reu|, +ud º|edd|uç o| ep|de|r|º |e+.|uç |+|çe
e|oº|ouº. I||º |º |er|u|ºceu| o| ºc+|d|uç.
1T8
S E C I | 0 N 9
8£NICN N£0FIASMS
AN0 h¥F£kFIASIAS
0IS0k0£kS 0F M£IAN0C¥I£S
£FI0£MI0I0C¥ AN0 £II0I0C¥
One of the most common acquiied new giowths
in Caucasians (most adults have about 20 nevi),
less common in blacks oi pigmented peisons,
and sometimes absent in peisons with ied haii
and maiked fieckling (skin phototype I).
kace Blacks and Asians have moie nevi on the
palms, soles, nail beds.
heredìty Common acquiied NMN occui in
family clusteis. Dysplastic melanocytic nevi
(DN) (see Section 12), which aie putative pie-
cuisoi lesions of malignant melanoma, occui in
viitually eveiy patient with familial cutaneous
melanoma and in 30-50% of patients with spo-
iadic nonfamilial piimaiy melanoma.
Suo £xposure A factoi in the induction of nevi
on the exposed aieas.
Sì¿oìIìcaoce Risk of melanoma is ielated to
the numbeis of NMN and to DN. In the lattei,
even if only a few lesions aie piesent.
CIINICAI MANIF£SIAII0N
0uratìoo aod £vo|utìoo oI Iesìoos NMN ap-
peai in eaily childhood and ieach a maximum
in young adulthood even though some NMN
may aiise in adulthood. Latei on theie is a
|e.ore|+uoc,||c ue.| (|\|), corrou|, c+||ed
¤¤|-º , +|e .e|, corrou, ºr+|| (·¹ cr), c||cur·
ºc|||ed, +cqu||ed p|çreu|ed r+cu|eº, p+pu|eº, o|
uodu|eº.
Corpoºed o| ç|oupº o| re|+uoc,||c ue.uº ce||º
|oc+|ed |u ||e ep|de|r|º, de|r|º, +ud, |+|e|,,
ºu|cu|+ueouº ||ººue.
I|e, +|e |eu|çu, +cqu||ed |uro|º +||º|uç +º ue.uº
ce|| c|uº|e|º +| ||e de|r+|·ep|de|r+| juuc||ou
( ·±¤:|·¤¤c| |!| ), |u.+d|uç ||e p+p|||+|, de|r|º
(:¤¤¤¤±¤! |!| ), +ud eud|uç ||e|| |||e c,c|e +º
!-·¤c| |!| W||| ue.uº ce||º |oc+|ed e\c|uº|.e|,
|u ||e de|r|º W|e|e, W||| p|oç|eºº|.e +çe, ||e|e
W||| |e ||||oº|º.
ACÇüIk£0 N£v0M£IAN0C¥IIC N£vI
giadual involution and fibiosis of lesions, and
most disappeai aftei the age of 60. In contiast,
DN continue to appeai thioughout life and aie
believed not to involute (see Section 12).
Skìo Symptoms NMN aie asymptomatic.
Howevei, NMN initially giow and giowth
is often accompanied by itching. If a lesion
¡ersísìenì|y itches oi is tendei, it should be
followed caiefully oi excised, since ¡ersísìenì
piuiitus may be an eaily indication of malig-
nant change.
CIASSIFICAII0N
NMN aie multiple (Fig. 9-1) and can be classi-
fied accoiding to theii state of evolution and
thus accoiding to the site of the clusteis of
nevus cells.
1. Juntìíona| me|anotyìít NMN : These aiise
at the deimal-epideimal junction, on the
epideimal side of the basement membiane;
in othei woids, they aie intiaepideimal
(Fig. 9-2).
2. Com¡ounJ me|anotyìít NMN : Nevus cells
invade the papillaiy deimis, and nevus cell
nests aie now found both intiaepideimally
and deimally (Fig. 9-3).
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 1T9
3. Derma| me|anotyìít NMN : These iepiesent
the last stage of the evolution of NMN.
°Diopping off " into the deimis is now
completed, and the nevus giows oi iemains
intiadeimal. With piogiessive age, theie will
be giadual fibiosis (Fig. 9-4).
Thus, melanocytic NMN undeigo the evolu-
tion fiom junctional compound deimal
NMN. Since the capacity of NMN cells to foim
melanin is gieatest when they aie located at
the deimal-epideimal junction (intiaepidei-
mally) and since NMN cells lose theii capacity
foi melanization, the fuithei they penetiate
into the deimis, the lessei is the intensity of
pigmentation with the inciease in the deimal
piopoition of the nevus. Puiely deimal NMN
aie theiefoie almost always without pigment.
In a simplified mannei, the clinical appeaiance
of NMN along this evolutionaiy path can be
chaiacteiized as follows: junctional NMN is
flat and daik, compound NMN is iaised and
daik, and deimal NMN is iaised and light. This
evolution also ieflects the age at which the dif-
feient types of NMN aie found. Junctional and
FICük£ 9-1 Mu|tìp|e NMN oo the shou|der oI a 32-year-o|d Iema|e. \oº| o| ||eºe ue.| +|e juuc||ou+|
|\|, ºore +|e º||ç|||, e|e.+|ed +ud ||uº corpouud |\|. |o|e |e|+||.e|, uu||o|r º|+pe +ud co|o| o| ||e
|eº|ouº. |ue |o d|||e|eu| de.e|opreu|+| º|+çeº ||e, +|e o| .+|,|uç º|/e |+uç|uç ||or ¹ |o + rr |u d|+re|e| +ud
||e, +|e |eçu|+| +ud |+.e + |e|+||.e|, uu||o|r º|+pe.
compound NMN aie usually seen in childhood
and thiough the teens, wheieas deimal NMN
stait manifesting in the thiid and fouith decade.
luoctìooa| Me|aoocytìc Nevoce||u|ar Nevì
Iesìoos Macule, oi only veiy slightly iaised
(Figs. 9-2, 33-13). Unifoim tan, biown, daik
biown, oi even black. Round oi oval with smooth,
iegulai boideis. Scatteied disciete lesions. Nevei
>1 cm in diametei; if >1 cm, the °mole" is a
congenital nevomelanocytic nevus, an atypical
nevus, oi a melanoma (see Section 12).
Compouod Me|aoocytìc Nevoce||u|ar Nevì
Iesìoos Papules oi small nodules (Fig. 9-3).
Daik biown, sometimes even black; dome-
shaped, smooth oi cobblestone-like suiface,
iegulai and shaiply defined boidei, sometimes
papillomatous oi hypeikeiatotic. Nevei >1 cm
in diametei; if >1 cm, the mole is a congenital
nevomelanocytic nevus, an atypical nevus, a
melanoma. Consistency eithei fiim oi soft.
Coloi may become mottled as piogiessive con-
veision into deimal NMN occuis. May have
haiis.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 180
0erma| Me|aoocytìc Nevoce||u|ar Nevì
Iesìoos Shaiply defined papule oi nodule.
Skin-coloied, tan oi flecks of biown, often
with telangiectasia. Round, dome-shaped (Fig.
9-4), smooth suiface, diametei <1 cm. Usually
not piesent befoie the second oi thiid decade.
Oldei lesions, mostly on the tiunk, may become
pedunculated and do not disappeai spontane-
ously. May be haiiy.
0ìstrìbutìoo Face, tiunk, extiemities, scalp.
Random, but some piedilection foi sun-
exposed aieas. Occasionally palmai and plantai,
in which case these NMN usually have the
appeaiance of junctional NMN.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
0ìa¿oosìs Made clinically. As foi all pigmented
lesions the ABCDE iule applies (see page 310).
In cases of doubt apply deimoscopy (epilumi-
nescence micioscopy), and if malignancy can-
not be excluded even by this pioceduie, excise
lesions with a naiiow maigin.
0ìIIereotìa| 0ìa¿oosìs Juntìíona| NMN : all
flat, deeply pigmented lesions. Solai lentigo,
flat atypical nevus, lentigo maligna. Com¡ounJ
NMN : all iaised pigmented lesions. Seboi-
iheic keiatosis, DN, small supeificial spieading
melanoma, eaily nodulai melanoma, pigmented
FICük£ 9-2 A-0 luoctìooa| NMN |eº|ouº +|e corp|e|e|, ||+| (â, 8 ) o| r|u|r+||, e|e.+|ed +º |u C. +ud
0. I|e, +|e º,rre|||c W||| + |eçu|+| |o|de| +ud, depeud|uç ou ||e º||u |,pe o| ||e |ud|.|du+|º, |+.e d|||e|eu|
º|+deº o| ||oWu |o ||+c| (0).
â 8
C 0
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 181
basal cell caicinoma, deimatofibioma, Spitz ne-
vus, blue nevus. Derma| NMN : all light tan oi
skin-coloied papules. Basal cell caicinoma, neu-
iofibioma, tiichoepithelioma, deimatofibioma,
sebaceous hypeiplasia.
MANAC£M£NI
Indications foi iemoval of acquiied melano-
cytic NMN aie the following:
Síìe : Lesions on the scalp (only if difficult to
follow and not a classic deimal NMN);
mucous membianes, anogenital aiea.
Crowì| : If theie is iapid change in size.
Co|or : If coloi becomes vaiiegated.
BorJer : If iiiegulai boideis aie piesent oi
develop.
Erosíons : If lesion becomes eioded without
majoi tiauma.
Sym¡ìoms : If lesion begins to ¡ersísìenì|y itch,
huit, oi bleed.
Dermosto¡y : If ciiteiia foi melanoma oi an
dysplastic nevus aie piesent oi appeai de
novo.
Melanocytic NMN ne·er become malignant
because of manipulation oi tiauma. In those
cases wheie this was claimed, the lesion was
initially a melanoma. If theie is an indi-
cation foi the iemoval of an NMN, the nevus
should always be excised foi histologic diag-
nosis and foi definite tieatment (paiticulaily
applicable to and decisive in iuling out con-
genital, dysplastic, oi blue nevi). Removal of
papillomatous, compound, oi deimal NMN
foi cosmetic ieasons by electiocauteiy iequiies
that a nevus be unequivocally diagnosed as
benign NMN and histology be peifoimed. If
an eaily melanoma cannot be excluded with
ceitainty, an excision foi histologic examina-
tion is obligatoiy but can be peifoimed with
naiiow maigins.
FICük£ 9-3 Compouod NMN uu||o|r|, p|çreu|ed p+pu|eº +ud ºr+|| dored uodu|eº. â. I|e |eº|ou |o
||e |e|| |º ||+||e| +ud |+u W||| + ro|e e|e.+|ed d+||e| ceu|e|, ||e |+|çe| |eº|ou (ou ||e ||ç||) |º o|de| +ud c|oco|+|e·
||oWu, ||e |e|| |eº|ou |º ,ouuçe| +ud |+º + p|edor|u+u||, juuc||ou+| corpoueu| +| ||e pe||p|e|,. 8. A |e+.||,
p|çreu|ed dore·º|+ped |eº|ou |u ||e e,e||oW. || |º º|+|p|, de||ued, uu||o|r|, ||+c|, ºroo|| +ud º||ç|||,
co|||eº|oue·|||e ºu||+ce, º|+|p|, +ud |eçu|+||, de||ued. || re+ºu|eº |eºº ||+u 5 rr.
8
â
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 182
â
FICük£ 9-4 0erma| me|aoocytìc NMN â. IWo dore·º|+ped, º|+|p|, de||ued |e|+||.e|, ºo|| |+u uodu|eº
ou ||e |e|| c|ee| +ud ||ç|| |+|e|+| r+ud||u|+| |eç|ou |u + o0·,e+|·o|d r+|e. I|eºe |eº|ouº We|e p|e.|ouº|, ruc|
d+||e| +ud |eºº e|e.+|ed. 8. A |+|çe| r+çu|||c+||ou o| + de|r+| |\|. I||º |eº|ou |º º|+|p|, de||ued, |+º + |edd|º|
co|o| W||| + ceu||+| |eçu|+| p|çreu|ed ºpo| W|e|e ||e ue.uº o|.|ouº|, |º º|||| corpouud |u u+|u|e. C. 0|d de|r+|
ue.uº ou ||e uppe| ||p o| + o5·,e+|·o|d Wor+u. I||º |eº|ou |º |e|+||.e|, |+|d, |+º + ºroo|| ºu||+ce +ud + p|u||º|
co|o|. I||º |eº|ou |º |u |eç|eºº|ou.
8 C
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 183
£FI0£MI0I0C¥
Oveiall pievalence 1%. Onset in the fiist thiee
decades. Occuis spontaneously and in patients
with vitiligo (18-26%). Also in patients with
metastatic melanoma (aiound metastatic le-
sions and aiound piimaiy melanoma). May
heiald vitiligo. All iaces, both sexes. Halo nevi
occui in siblings and in those with a family his-
toiy of vitiligo.
FAIh0C£N£SIS
Immunologic phenomena, both humoial and
cellulai, aie iesponsible foi the dynamic changes
that eventually lead to nevus involution.
CIINICAI MANIF£SIAII0N
Theie aie thiee stages: (1) Development (in
months) of white halo aiound pieexisting
NMN; halo may be pieceded by faint eiythema;
(2) disappeaiance (months to yeais) of NMN;
and (3) iepigmentation (months to yeais) of
halo.
Fh¥SICAI £XAMINAII0N
Skìo Iesìoos Papulai biown NMN (<5 mm)
with oval oi iound halo of shaiply maiginated
hypomelanosis (Figs. 9-5 and 9-6 À ). The
NMN is tenìra||y located. These usually show
depigmentation with wood lamp examination.
Scatteied disciete lesions (1 to >30) mostly on
the tiunk, but in geneial the same distiibution
as of NMN (Fig. 9-5). May begin with eiythema
aiound NMN (Fig. 9-6 B ).
Specìa| Forms
Congenital halo NMN occui iaiely.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
If clinical findings atypical: the nevus has vaii-
egation of coloi and/oi iiiegulai boideis, con-
fiim histologically and exclude melanoma.
"ha|o" 0epì¿meotatìoo arouod 0ther
Iesìoos Can occui aiound blue nevus, con-
genital NMN, Spitz juvenile nevus, veiiuca
plana, piimaiy melanoma, melanoma metas-
tases, deimatofibioma, neuiofibioma.
FICük£ 9-5 ha|o me|aoocytìc
NMN oo the back oI a 22-year-
o|d Iema|e I|e|e +|e ||.e |+|o
ue.|, +|| W||| + p|çreu|ed do|·|||e
ceu||+| juuc||ou+| o| corpouud |\|
ºu||ouuded |, + |,po· o| +re|+uo||c
|+|o. I|e +||oW |ud|c+|eº oue |eº|ou
W|e|e ||e ceu||+| ue.uº |+º cor·
p|e|e|, |eç|eººed, ||e |edd|º| co|o|
|ud|c+|eº |e|+uç|ec|+º|+.
Au |\| ||+| |º euc||c|ed |, + |+|o o| |eu|ode|r+
o| dep|çreu|+||ou. I|e |eu|ode|r+ |º |+ºed ou
+ dec|e+ºe o| re|+u|u |u re|+uoc,|eº +ud/o|
d|º+ppe+|+uce o| re|+uoc,|eº +| ||e de|r+|·ep|·
de|r+| juuc||ou.
A W|||e |+|o +|ouud + |\| |ud|c+|eº |eç|eºº|ou
n+|o ue.| roº| o||eu uude|ço ºpou|+ueouº |u·
.o|u||ou, o||eu W||| |eç|eºº|ou o| ||e ceu||+||,
|oc+|ed p|çreu|ed ue.uº.
n+|o |\| r+, |ud|c+|e |uc|p|eu| .|||||ço.
',¤¤¤,¤ . 'u||ou |eu|ode|r+ +cqu|º||ur ceu·
||||uçur.
hAI0 N£v0M£IAN0C¥IIC N£vüS |C|·9 . 2¹o.9

|C|·¹0 . |22·\312!/0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 184
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Junctional deimal oi com-
pound nevus suiiounded by lymphocytic in-
filtiate (lymphocytes and histiocytes) aiound
and between nevus cells. Nevus cells develop
evidence of cell damage undeigo apoptosis, and
disappeai. Halo shows deciease oi total absence
of melanin and melanocytes.
MANAC£M£NI
Reassuiance. Excision if the featuies of the ne-
vus aie atypical: vaiiegation of coloi, iiiegulai
boideis.
FICük£ 9-6 ha|o me|aoocytìc NMN â. |+|çe| r+çu|||c+||ou o| + |+|o |\|. I|e ue.uº |º + juuc||ou+|
|\| (corp+|e W||| ||ç. 9·2) ||+| |º ºu||ouuded |, + |,pore|+uo||c (+|roº| W|||e) |+|o. 8. 'e.e|+| |+u juuc||ou+|
|\| ||+| +|e ºu||ouuded |, +u e|,||er+|ouº |+|o. I||º |º ||e e+||, º|+çe o| |+|o de.e|opreu|. I|e
e|,||er+|ouº ||r W||| |+|e| |e |ep|+ced |, + |,pore|+uo||c |+|o.
â 8
£FI0£MI0I0C¥
0oset In childhood and late adolescence.
Equal sex distiibution.
varìaots Thiee types: common blue nevus,
cellulai blue nevus, combined blue nevus-
nevomelanocytic nevus.
FAIh0C£N£SIS
Ectopic accumulations of melanin-pioducing
melanocytes (not nevus cells) in the deimis
deiived fiom melanoblasts that became aiiested
duiing theii migiation fiom neuial ciest to sites
in the skin.
A ||ue ue.uº |º +u +cqu||ed, |||r, d+||·||ue |o
ç|+,·|o·||+c|, º|+|p|, de||ued p+pu|e o| uodu|e
|ep|eºeu||uç + |oc+||/ed p|o|||e|+||ou o| re|+u|u·
p|oduc|uç !-·¤c| re|+uoc,|eº.
A ||ue ue.uº |º |eu|çu. 'o·c+||ed ce||u|+| ||ue
ue.| +|e |+|çe| +ud |+.e .e|, |+|e |eudeuc, |o
|ecore r+||çu+u|.
',¤¤¤,¤º . B|ue ueu|oue.uº, de|r+| re|+uoc,·
|or+.
8Iü£ N£vüS |C|·9 . 2¹o.9

|C|·¹0 . |22. \3130
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 185
CIINICAI MANIF£SIAII0N
Neaily always asymptomatic, occasion-
ally of cosmetic concein; often feaied to be
melanoma.
Fh¥SICAI £XAMINAII0N
Skìo Iesìoos Papules to nodules, blue, blue-
giay, blue-black, usually <10 mm in diametei
(Figs. 9-7 and 9-8 À ). Ce||u|ar ||ue ne·í aie laigei
(1-3 cm) (Fig. 9-8 B ). Occasionally have taiget-
like pattein of pigmentation. Usually iound to
oval. Com|íneJ ||ue ne·us - NMN : blue-biown
oi blue-black with a lightei iim.
SItes v] PredI|ectIvn Most commonly located
on the doisa of hands (Fig. 9-8 À ) oi feet (50%),
but many occui in the face (Fig. 9-7); cellulai
blue nevi occui on the buttocks, lowei back,
scalp (Fig. 9-8 B ), and face.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Melanin-containing wavy
deimal melanocytes with long thin dendiites
giouped in iiiegulai bundles admixed with
melanin-containing maciophages in the up-
pei oi middle deimis: excessive fibious tissue
pioduction in uppei ieticulai deimis. Ce||u-
|ar ||ue ne·us : in addition to spindle-shaped
melanocytes, epithelioid nevus cells in deimis
and subcutaneous fat in nests and neuioid
foims. Com|íneJ ||ue ne·us - NMN : combi-
nation of blue nevus and compound NMN.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Usually made on clinical findings, at times
confiimed by excision and deimatopathologic
examination to iule out nodulai melanoma.
8|ue[Cray Fapu|e Deimatofibioma, glomus
tumoi, piimaiy (nodulai) oi metastatic
melanoma, pigmented spindle cell (Spitz) nevus,
tiaumatic tattoo, angiokeiatoma, pigmented
basal cell caicinoma.
C0ükS£ AN0 Fk0CN0SIS
Most iemain unchanged. Malignant melanoma
iaiely develops in te||u|ar blue nevi.
MANAC£M£NI
Blue nevi <10 mm in diametei and stable foi
many yeais usually do not need excision. Sud-
den appeaiance oi change of an appaient blue
nevus waiiants suigical excision and deimat-
opathologic examination. Cellulai blue nevi
( 1-3 cm; Fig. 9-8B) aie usually excised to iule
out melanoma.
FICük£ 9-T 8|ue oevus I|e|e +|e |ou| |+u juuc||ou+| |\| +ud oue ||u|º|·||+c| |ouud |eº|ou ou ||e c|ee|
o| + ¹1·,e+|·o|d ç|||. |u cou||+º| |o ||e juuc||ou+| |\|º ||e ||ue ue.uº |º p+|p+||e W||| + |e|+||.e|, ||ç| couº|º·
|euc, +ud upou de|roºcop, W||| +ppe+| +º +u |||·de||ued uu||o|r|, ||u|º| |eº|ou deep |u ||e de|r|º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 186
FICük£ 9-8 8|ue oevus aod ce||u|ar b|ue oevus â. I||º ||ue ue.uº |+º |eçu|+| |o|de|º |u| |º uo| c||·
cu|+| +ud |º ºo||d|, ||ue·||+c| |u co|o|. I|e ep|de|r|º |º ºroo||, |ud|c+||uç ||+| ||e |eº|ou |º |u ||e de|r|º. I|e
couº|º|euc, |º |uc|e+ºed +ud ||e r+|ç|uº +|e We|| de||ued. ||||e|eu||+| d|+çuoº|º ruº| |uc|ude uodu|+| re|+uor+.
|| ||e |eº|ou |+º |eeu p|eºeu| |o| ,e+|º, + ||opº, |º uo| ueceºº+|,. || ||e |eº|ou W+º uo|ed ou|, + |eW rou||º +ço,
e\c|º|ou ||opº|eº +|e |equ||ed |o |u|e ou| uodu|+| re|+uor+. noWe.e|, de|roºcop, ç|e+||, |+c||||+|eº ||e c||u|c+|
d|||e|eu||+| d|+çuoº|º º|oW|uç uoue o| ||e |e+|u|eº o| uodu|+| re|+uor+ +ud W||| ||e|e|o|e |eude| +u e\c|º|ou+|
||opº, uuueceºº+|,. 8. I||º ce||u|+| ||ue ue.uº +ppe+|ed +º |Wo |+|çe, ||u|º|·||+c| uodu|eº ou ||e ºc+|p. A||e|
e\c|º|ou, ||º|o|oç, º|oWed ||+| ||e, We|e cou||çuouº +ud ||uº |ep|eºeu|ed oue º|uç|e |eº|ou. Ce||u|+| ||ue ue.|
+|e ruc| |+|çe| +ud º|ou|d +|W+,º |e e\c|ºed |o |u|e ou| re|+uor+, W||c|, +||e|| |+|e|,, c+u de.e|op |u ||eºe
|eº|ouº.
â 8
A |+||e| corrou re|+uoc,||c |eº|ou ||+| couº|º|º
o| + ||ç|| ||oWu p|çreu|ed r+cu|e .+|,|uç ||or
+ |eW ceu||re|e|º |o + .e|, |+|çe +|e+ (>¹5 cr),
+ud r+u, d+|| ||oWu ºr+|| r+cu|eº (2-! rr)
o| p+pu|eº ºc+||e|ed |||ouç|ou| ||e p|çreu|ed
|+c|ç|ouud (||ç. 9·9 1 ). I|e p|çreu| |u ||e
r+cu|+| |+c|ç|ouud r+, |e ºo |+|u| ||+| || c+u
|e |ecoçu|/ed ou|, uude| wood ||ç|| (||ç. 9·9 3 ).
I|e p+||o|oç, o| ||e |+c|ç|ouud o| ||e r+cu|+|
p|çreu|ed |eº|ou |º ||e º+re +º |eu||ço º|rp|e\,
|.e., |uc|e+ºed uur|e|º o| re|+uoc,|eº, W|||e
||e ||+| o| |+|ºed |eº|ouº ºc+||e|ed |||ouç|ou| +|e
e|||e| juuc||ou+| o| corpouud, |+|e|,, ||eºe +|e
||.
I|e |eº|ouº +|e uo| +º corrou +º juuc||ou+| o|
corpouud |\| |u| +|e uo| +| +|| |+|e. |u oue
ºe||eº, ||e ue.uº ºp||uº W+º p|eºeu| |u !° o|
W|||e p+||eu|º.
\+||çu+u| re|+uor+ .e|, |+|e|, +||ºeº |u ||eºe
|eº|ouº.
N£vüS SFIIüS |C|·9 . 2¹o.9

|C|·¹0 . |22
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 18T
â
8
FICük£ 9-9 Nevus spì|us â. I||º d+|| ||oWu p|çreu|ed r+cu|e re+ºu||uç +|ou| ¹0 cr +|ouç ||e |ouç
+\|º |º peppe|ed W||| r+u, ºr+||, d+|| ||oWu |o ||+c| r+cu|eº +ud p+pu|eº. 8. I||º |º +|ºo ue.uº ºp||uº |u| ||e
r+cu|+| |+c|ç|ouud |º ou|, º||ç|||, p|çreu|ed ºo ||+| || W||| |e |e.e+|ed ou|, uude| wood ||ç||. I|e |eº|ou |º pep·
pe|ed W||| r+u, ºr+|| d+|| ||oWu r+cu|eº +ud ||+| p+pu|eº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 188
FICük£ 9-10 Spìtt oevus â. ||u| dore·º|+ped uodu|e ou ||e c|ee| o| + ,ouuç Wor+u, de.e|op|uç
+||up||, W||||u ||e p|e.|ouº ¹2 rou||º, ||e |eº|ou c+u |e r|º|+|eu |o| + |er+uç|or+. 8. ||çreu|ed 'p||/ ue.uº.
A ||+c| p+pu|e ºu||ouuded |, + |+u r+cu|+| |eç|ou (|eu||ç|uouº) de.e|oped W||||u + |eW rou||º ou ||e |+c| o| +
,ouuç |er+|e, +º ºuc| + |eº|ou c+uuo| |e d|º||uçu|º|ed ||or + uodu|+| re|+uor+, ||e |eº|ou W+º e\c|ºed +ud
||e d|+çuoº|º cou|||red ||º|o|oç|c+||,
â
8
'p||/ ue.uº |º + |eu|çu, dore·º|+ped, |+|||eºº,
ºr+|| (·¹ cr |u d|+re|e|) uodu|e, roº| o||eu
p|u| o| |+u (||ç. 9·¹01). I|e|e |º o||eu + ||º|o|, o|
|eceu| |+p|d ç|oW||.
|uc|deuce |º ¹.+.¹00,000 (Auº||+||+). || occu|º +|
+|| +çeº. A ||||d o| ||e p+||eu|º +|e c|||d|eu ·¹0
,e+|º, + ||||d +|e ¹0-20 ,e+|º o|d, +ud + ||||d
+|e >20, |+|e|, ºeeu |u pe|ºouº +0 ,e+|º. |-·
º·¤¤º +||ºe W||||u rou||º. I|e, +|e p+pu|eº o|
dore·º|+ped o| |e|+||.e|, ||+| uodu|eº, |ouud,
We||·c||urºc|||ed, ºroo||·|opped, +ud |+|||eºº.
I|e, +|e + uu||o|r p|u| (||ç. 9·¹0 1 ), |+u, ||oWu,
d+|| ||oWu, o| e.eu ||+c| (||ç. 9·¹0 3 ), +|e |||r,
+ud uºu+||, d|º||||u|ed ou ||e |e+d +ud uec|.
|·||-·-¤|·c| !·c¸¤¤º·º |uc|udeº +|| p|u|, |+u, o|
d+|||, p|çreu|ed p+pu|eº. p,oçeu|c ç|+uu|or+,
|er+uç|or+, ro||uºcur cou|+ç|oºur, ju.eu||e
\+u||oç|+uu|or+, r+º|oc,|or+, de|r+|o||·
||or+, |\|, ||, uodu|+| re|+uor+.
|-·¤c|¤¤c||¤|¤¸, couº|º|º o| |,pe|p|+º|+ o| ||e
ep|de|r|º +ud o| re|+uoc,|eº, d||+|+||ou o| c+p||·
|+||eº. I|e|e +|e +dr|\ed |+|çe ep|||e||o|d ce||º,
|+|çe ºp|ud|e ce||º W||| +|uud+u| c,|op|+ºr, +ud
occ+º|ou+| r||o||c ||çu|eº. I|e|e +|e ºore||reº
||/+||e c,|o|oç|c p+||e|uº. ueº|º o| |+|çe ce||º
e\|eud ||or ||e ep|de|r|º ('|+|u|uç doWu¨)
|u|o ||e |e||cu|+| de|r|º +º |+ºc|c|eº o| ce||º |o|r
+u '|u.e||ed |||+uç|e,¨ W||| ||e |+ºe |,|uç +| ||e
de|r+|·ep|de|r+| juuc||ou +ud ||e +pe\ |u ||e
|e||cu|+| de|r|º.
n|º|o|oç|c e\+r|u+||ou ruº| |e doue |o cou|||r
||e c||u|c+| d|+çuoº|º. E\c|º|ou |u ||º eu|||e|, |º |r·
po||+u| |ec+uºe ||e coud|||ou |ecu|º |u ¹0-¹5°
o| +|| c+ºeº |u |eº|ouº ||+| |+.e uo| |eeu e\c|ºed
corp|e|e|,. 'p||/ ue.| +|e |eu|çu, |u| ||e|e c+u
|e + ||º|o|oç|c º|r||+|||, |o re|+uor+ +ud ||e
||º|op+||o|oç|c d|+çuoº|º |equ||eº ||e |e|p o| +u
e\pe||euced de|r+|op+||o|oç|º|.
'p||/ |uro|º p|o|+||, do uo| uºu+||, |u.o|u|e,
+º do corrou +cqu||ed |\| ue.|. noWe.e|,
ºore |eº|ouº |+.e |eeu o|ºe|.ed |o ||+uº|o|r
|u|o corrou corpouud |\|, +ud ºore uu·
de|ço ||||oº|º +ud |u |+|e º|+çeº r+, |eºer||e
de|r+|o||||or+º.
',¤¤¤,¤º . ||çreu|ed +ud ep|||e||o|d ºp|ud|e
ce|| ue.uº. \e+|º +ço ||eºe We|e c+||ed 'ju.eu||e
re|+uor+.¨
SFIII N£vüS |C|·9 . 2¹o.9

|C|·¹0 . |22·\3112
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 189
FICük£ 9-11 Moo¿o|ìao spot A |+|çe ç|+,·
||ue r+cu|+| |eº|ou |u.o|.|uç ||e eu|||e |ur|oº+c|+|
+ud ç|u|e+| +|e+ +ud ||e |e|| |||ç| |u + |+|, ||or '||
|+u|+. A|||ouç| \ouço||+u ºpo|º +|e corrou |u
Aº|+uº, ||e p+|eu|º o| |||º |+|, We|e +|+|red |ec+uºe
||e |eº|ou W+º ºo |+|çe.
FICük£ 9-12 Moo¿o|ìao spots \u|||p|e, |||·de||ued,
||u|º| |eº|ouº +|e ºc+||e|ed ou ||e |+c| o| |||º c|||d o|
l+p+ueºe deºceu|. I|e, We|e p|eºeu| +| |||||. \oº| o|
||eºe |eº|ouº d|º+ppe+|ed |+|e| |u c|||d|ood.
I|eºe couçeu||+| ç|+,·||ue r+cu|+| |eº|ouº +|e
c|+|+c|e||º||c+||, |oc+|ed ou ||e |ur|oº+c|+| +|e+
(||ç. 9·¹¹) |u| c+u +|ºo occu| ou ||e |+c|, ºc+|p,
o| +u,W|e|e ou ||e º||u. I|e|e |º uºu+||, + º|uç|e
|eº|ou, |u| |+|e|,, ºe.e|+| ||uuc+| |eº|ouº c+u |e
p|eºeu| +| ||||| (||ç. 9·¹2).
I|e uude||,|uç p+||o|oç, |º d|ºpe|ºed ºp|ud|e·
º|+ped re|+uoc,|eº W||||u ||e de|r|º (de|r+|
re|+uoc,|oº|º). \e|+uoc,|eº +|e uo| uo|r+||,
p|eºeu| |u ||e de|r|º, +ud || |º |e||e.ed ||+| ||eºe
ec|op|c re|+uoc,|eº |ep|eºeu| p|çreu| ce||º ||+|
|+.e |eeu |u|e||up|ed |u ||e|| r|ç|+||ou ||or ||e
ueu|+| c|eº| |o ||e ep|de|r|º.
\ouço||+u ºpo|º r+, d|º+ppe+| |u e+||, c|||d·
|ood, |u cou||+º| |o ue.uº o| 0|+ (ºee ||ç.
9·¹! 1 ).
Aº ||e |e|r !¤¤¸¤|·c¤ |rp||eº, ||eºe |eº|ouº
+|e |ouud +|roº| +|W+,º (99-¹00°) |u |u|+u|º o|
Aº|+||c +ud |+||.e Are||c+u o||ç|u, |oWe.e|, ||e,
|+.e |eeu |epo||ed |u ||+c| +ud, |+|e|,, |u W|||e
|u|+u|º.
|o re|+uor+º |+.e |eeu |epo||ed |o occu| |u
||eºe |eº|ouº.
|u Aº|+uº.
M0NC0IIAN SF0I |C|·9 . 151.!!
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 190
I||º p|çreu|+|, d|ºo|de| |º .e|, corrou |u Aº|+u
popu|+||ouº +ud |º º+|d |o occu| |u ¹° o| de|r+·
|o|oç|c ou|p+||eu|º |u l+p+u. || |+º |eeu |epo||ed
|u E+º| |ud|+uº, ||+c|º, +ud, |+|e|,, W|||eº.
 I|e p|çreu|+||ou, W||c| c+u |e qu||e ºu|||e o|
r+||ed|, d|º||çu||uç, couº|º|º o| + ro|||ed, duº|,
+dr|\|u|e o| ||ue +ud ||oWu |,pe|p|çreu|+||ou o|
||e º||u. I|e p|çreu|+||ou roº||, |u.o|.eº ||e º||u
+ud rucouº rer||+ueº |uue|.+|ed |, ||e |||º| +ud
ºecoud ||+uc|eº o| ||e |||çer|u+| ue|.e (||ç. 9·¹!).
I|e ||ue |ue |eºu||º ||or ||e p|eºeuce o| ec|op|c
re|+uoc,|eº |u ||e de|r|º. || c+u occu| |u ||e
|+|d p+|+|e +ud |u ||e coujuuc||.+e (||ç. 9·¹! 3 ),
ºc|e|+e, +ud |,rp+u|c rer||+ueº.
|e.uº o| 0|+ r+, |e |||+|e|+| (||ç. 9·¹! C ). || r+,
|e couçeu||+| |u| |º uo| |e|ed||+|,, ro|e o||eu ||
+ppe+|º |u e+||, c|||d|ood o| du||uç pu|e||, +ud
|er+|uº |o| |||e, |u cou||+º| |o ||e \ouço||+u ºpo|,
W||c| r+, d|º+ppe+| |u e+||, c|||d|ood.
I|e+|reu| W||| |+ºe|º |º +u e||ec||.e rod+|||, |o|
|||º d|º||çu||uç d|ºo|de|.
\+||çu+u| re|+uor+ c+u occu| |u| |º |+|e.
*|u Aº|+uº.
N£vüS 0F 0IA |C|·9 . 2¹o.9

|C|·¹0 . |22
â
FICük£ 9-13 Nevus oI 0ta â. I|e|e |º +u |||·de||ued, ro|||ed, duº|,, ç|+, |o ||u|º| |,pe|p|çreu|+||ou |u
||e |eç|ouº ºupp||ed |, ||e |||º| +ud ºecoud ||+uc|eº o| ||e |||çer|u+| ue|.e. I|e |eº|ou W+º uu||+|e|+| +ud ||e|e
W+º +|ºo |,pe|p|çreu|+||ou o| ||e ºc|e|+.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 191
8
FICük£ 9-13 Nevus oI 0ta (Cootìoued) 8. B|oWu|º| |o ç|+, |,pe|p|çreu|+||ou ou ||e uppe| +ud |oWe|
||dº +ud + ºpec||ed |,pe|p|çreu|+||ou o| ||e ºc|e|+ |u + |+||º|+u| |o,. I|e |eº|ou |º uu||+|e|+|. C. B||+|e|+| ue.uº o|
0|+ W||| |u.o|.ereu| o| ||e ºc|e|+e |u + l+p+ueºe c|||d.
C
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 192
I|e p|eºeu| ||u+|, ||o|oç|c c|+ºº|||c+||ou d|º·
||uçu|º|eº |e|Weeu .+ºcu|+| |uro|º +ud .+ºcu|+|
r+||o|r+||ouº. I|e |+||e| +|e ºu|c|+ºº|||ed +cco|d·
|uç |o ||e º||uc|u|+| corpoueu|º |u|o c+p|||+|,,
.euouº, |,rp|+||c, +||e||+|, o| cor||ued |o|rº.
lcº:±|c· |±¤¤·º (e.ç., |er+uç|or+º) º|oW eu·
do||e||+| |,pe|p|+º|+, W|e|e+º ¤c||¤·¤c|·¤¤º
|+.e + uo|r+| eudo||e||+| |u|uo.e|.
ner+uç|or+º o| |u|+uc, +|e uo| p|eºeu| +| |||||
|u| +ppe+| poº|u+|+||,, ç|oW |+p|d|, du||uç ||e
|||º| ,e+| (p|o|||e|+||uç p|+ºe), uude|ço º|oW
ºpou|+ueouº |eç|eºº|ou du||uç c|||d|ood (|u.o|u·
||ou p|+ºe), +ud |er+|u º|+||e ||e|e+||e|.
\+ºcu|+| r+||o|r+||ouº +|e e||o|º o| ro|p|oçeu·
eº|º +ud +|e p|eºured |o occu| du||uç |u||+u|e|·
|ue |||e. \oº| +|e p|eºeu| +| |||||, ||ouç| ºore do
uo| +ppe+| uu||| ,e+|º |+|e|. 0uce r+u||eº| ||e,
ç|oW p|opo|||ou+||,, |u| eu|+|çereu| c+u occu|
+º + |eºu|| o| .+||ouº |+c|o|º.
Bo|| .+ºcu|+| |uro|º +ud r+||o|r+||ouº c+u |e
ºep+|+|ed |u|o º|oW·||oW o| |+º|·||oW |,peº.
C|+ºº|||c+||ou o| .+ºcu|+| |uro|º +ud r+||o|r+·
||ouº |º º|oWu |u I+||e 9·¹ +ud ||e d|º||uçu|º||uç
|e+|u|eº o| .+ºcu|+| |uro|º +ud .+ºcu|+| r+||o|·
r+||ouº +|e º|oWu |u I+||e 9·2.
vASCüIAk IüM0kS AN0 MAIF0kMAII0NS
IA8I£ 9-1 C|assification of Vascu|ar Anoma|ies
vascu|ar Iumors vascu|ar Ma|Iormatìoos
ner+uç|or+ C+p|||+|,
ner+uç|or+ o| |u|+uc, C+p|||+|, r+||o|r+||ou (po||·W|ue º|+|u)
Couçeu||+| Ie|+uç|ec|+º|+ (|e|ed||+|, |eu|çu |e|+uç|ec|+º|+, eººeu||+|
· k+p|d|, |u.o|u||uç couçeu||+| |e|+uç|ec|+º|+)
|er+uç|or+ ne|ed||+|, |ero|||+ç|c |e|+uç|ec|+º|+
· |ou|u.o|u||uç couçeu||+| C+p|||+|, r+||o|r+||ou·+||e||o.euouº r+||o|r+||ou
|er+uç|or+ '|u|çe·we|e| º,ud|ore
ner+uç|oeudo||e||or+º \euouº
K+poº||o|r |er+uç|oeudo||e||or+ \euouº r+||o|r+||ou
Iu||ed +uç|or+ |+r|||+| |o|r. Cu|+ueorucoº+| .euouº r+||o|r+||ou
Auç|oº+|cor+ C|oru.euouº r+||o|r+||ou
 B|ue |u||e| ||e| ue.uº o| Be+u º,ud|oreº
|,rp|+||c
 |,rp|+||c r+||o|r+||ou
 |||r+|, |,rp|oeder+º
A||e||+|
 A||e||o.euouº r+||o|r+||ou
 C+p|||+|, r+||o|r+||ou·+||e||o.euouº r+||o|r+||ou
 A||e||o.euouº ||º|u|+
',ud|or|c r+||o|r+||ouº
 '|oW·||oW
 · K||ppe|·I|eu+uu+, º,ud|ore (c+p|||+|,·|,rp|+||co.euouº
r+||o|r+||ou)
 · \+|||ucc| º,ud|ore
 |+º|·||oW
· |+||eº we|e| º,ud|ore
'ou|ce. |\ Boou, +ud \ \|||u||+, |u K wo||| e| +| (edº). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, 1|| ed. |eW \o||, \cC|+W·n|||, 2003,
pp ¹o5¹-¹ooo.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 193
vASCüIAk IüM0kS
IA8I£ 9-2 0istin¿uishin¿ |eatures of Vascu|ar Iumors (heman¿iomas) and Vascu|ar Na|formations
T0mors Na|Iormat|oos
||eºeuce +| ||||| uºu+||, poº|u+|+|, !0° u+ºceu|, ¹00° (p|eºur+||,), uo|
|+|e|, |u|| ç|oWu +|W+,º o|.|ouº
\+|e.|er+|e |+||o ¹.!-¹.5 ¹.¹
|uc|deuce ¹-¹2.o° +| |||||, ¹0-¹2° 0.!-0.5° po||·W|ue º|+|u
+| ¹ ,e+|
|+|u|+| ||º|o|, ||+ºeº. p|o|||e|+||uç, |u.o|u||uç, ||opo|||ou+|e ç|oW||,
+ud |u.o|u|ed c+u e\p+ud
Ce||u|+| Eudo||e||+| |,pe|p|+º|+ |o|r+| eudo||e||+| |u|uo.e|
'|e|e|+| c|+uçeº 0cc+º|ou+| r+ºº e||ec| ou '|oW·||oW. d|º|o|||ou,
+dj+ceu| |oue, |+|e |,pe|||op|,, o|
|,pe|||op|, |,pe|p|+º|+
|+º|·||oW. deº||uc||ou,
d|º|o|||ou, o| |,pe|||op|,
'ou|ce. ' \||ue|||·C|e.e||u|, lB \u||||eu, |u |\ ||eed|e|ç e| +| (edº). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, o|| ed. |eW \o||,
\cC|+W·n|||, 200!, pp ¹002-¹0¹9.
h£MANCI0MA 0F INFANC¥ (hI)
|C|·9 . 151.!2

|C|·¹0 . |¹3.0·\9¹!¹
(Foimeily stiawbeiiy, cheiiy, capillaiy heman-
gioma.)
£FI0£MI0I0C¥
HI is the most common tumoi of infancy. The
incidence in newboins is between 1 and 2.5%;
in white childien by 1 yeai of age it is 10%.
Females moie affected than males by a 3 to 1
iatio.
£II0I0C¥ AN0 FAIh0C£N£SIS
HI is a localized piolifeiative piocess of an-
gioblastic mesenchyme. It iepiesents a clonal
expansion of endothelial cells that may iesult
fiom somatic mutations of genes iegulating
endothelial cell piolifeiation.
CIINICAI MANIF£SIAII0N
The initial piolifeiative phase lasts fiom 3 to
9 months, sometimes moie. HIs usually enlaige
iapidly duiing the fiist yeai. In a subsequent
phase of involution the HI iegiesses, and this
occuis giadually ovei 2 to 6 yeais and is usually
complete by the age of 10. Involution vaiies
gieatly between individuals and is not coiie-
lated with size, location, oi appeaiance of the
lesion.
Skìo Iesìoos Soft, biight ied to deep puiple,
compiessible. On diascopy, does not blanch
completely. Nodule oi plaque, 1-8 cm (Figs.
9-14À, 9-15 À ). With the onset of spontaneous
iegiession, a white-to-giay aiea appeais on the
suiface of the cential pait of the lesion (Fig. 9-
15À ). Ulceiation may occui.
DIstrIhutIvn Lesions aie usually solitaiy and
localized oi extend ovei an entiie iegion (Fig.
9-16). Head and neck 50%, tiunk 25%. Face,
tiunk, legs, oial mucous membiane.
SF£CIAI Fk£S£NIAII0NS
0eep hemao¿ìoma (Foimeily, caveinous
hemangioma.) In the lowei deimis and sub-
cutaneous fat. Localized, fiim iubbeiy mass of
bluish oi noimal skin coloi with telangiectases
in oveilying skin (Fig. 9-17). Can be combined
with supeificial hemangioma (Fig. 9-15 À ). Does
not involute as well as supeificial type.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 194
Mu|tìp|e hIs Multiple small (<2 cm), cheiiy-
ied papulai lesions involving skin alone ( |enígn
tuìaneous |emangíomaìosís ) oi skin and intei-
nal oigans ( Jí[[use neonaìa| |emangíomaìosís ).
Coo¿eoìta| hemao¿ìomas These develop in
uteio and aie subdivided into iapidly involuting
congenital hemangiomas (RICH) and noninvo-
luting congenital hemangiomas (NICH). They
piesent as violaceous tumois with oveilying
telangiectasia with laige veins in peiipheiy oi as
ied-violaceous plaques invading deepei tissues.
NICH aie fast-flow hemangiomas iequiiing
suigeiy.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Piolifeiation of endothe-
lial cells in vaiious amounts in the deimis
and/oi subcutaneous tissue; theie is usually
moie endothelial piolifeiation in the supeificial
type and little in the deep angiomas. GLUT-1
immunoieactivity is found in all hemangiomas
but not in vasculai malfoimations.
0IACN0SIS
Made on clinical findings and MRI; Dopplei
and aiteiiogiaphy to demonstiate fast flow. De-
teimine GLUT-1 immunoieactivity to iule out
vasculai malfoimation.
C0ükS£ AN0 Fk0CN0SIS
HIs spontaneously involute by the fifth yeai,
with some few peicent disappeaiing only by age
10 (Figs. 9-14 B and 9-15 B ). Theie is viitually
no iesidual skin change at the site in most le-
sions (80%); in the iest theie is atiophy, dep-
igmentation, telangiectasia, and scaiiing. HIs
may, howevei, pose a consideiable pioblem
duiing the giowth phase when they inteifeie
with vital functions, such as obstiuction of vi-
sion (Fig. 9-16) oi of laiynx, nose, oi mouth.
Deepei lesions, especially those involving mu-
cous membianes, may not involute completely.
Synovial involvement may be associated with
hemophilia-like aithiopathy. Special foims of
HI, ìu[ìeJ angíomas and Ka¡osí[orm |eman-
gíoenJoì|e|íoma may have platelet entiapment,
thiombocytopenia (Kasabach-Meiiitt syn-
diome), and even disseminated intiavasculai
coagulation. Raiely, moibidity associated with
HI occuis secondaiy to hemoiihage oi high-
output heait failuie.
MANAC£M£NI
Each lesion must be judged individually iegaid-
ing the decision to tieat oi not to tieat and
the selection of a tieatment mode. Systemic
tieatment is difficult, iequiies expeiience, and
should be peifoimed by an expeit. Suigical and
medical inteiventions include continuous wave
oi pulsed dye lasei, ciyosuigeiy, intialesional
and systemic high-dose glucocoiticoids, intei-
feion (IFN- ), and piopanolol. Foi the ma-
joiity of HIs active noninteivention is the best
appioach because spontaneous iesolution gives
the best cosmetic iesults (Figs. 9-14B, 9-15B).
Tieatment is indicated in about a quaitei of HIs
(5% that ulceiate; 20% that obstiuct vital stiuc-
tuies, i.e., eyes, eais, laiynx) and in the <1% that
aie life thieatening.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 195
â 8
FICük£ 9-14 hemao¿ìoma oI ìoIaocy â. I||º |||ç|| |ed uodu|+| p|+que |u +u |u|+u| o| A|||c+u e\||+c||ou
|º |||ç||eu|uç |o ||e p+|eu|º, +ud c+u||ou |º ueeded |o p|e.eu| ºc+|||uç ||or ||e ||e+|reu| ||ºe||. '|uce roº| o|
||eºe |eº|ouº d|º+ppe+| ºpou|+ueouº|, W||| ou|, 20° º|oW|uç |eº|du+| +||op|, o| dep|çreu|+||ou, + W+||·+ud·ºee
º||+|eç, |º |ecorreuded. 8. I|e º+re |eº|ou +||e| ! ,e+|º. I|e |er+uç|or+ |+º |+ded ºpou|+ueouº|,, +ud ||e|e
|º ou|, º||ç|| |eº|du+| +||op|,.
â 8
FICük£ 9-15 hemao¿ìoma oI ìoIaocy â. I||º |eº|ou ou ||e uoºe couº|º|º o| + ºupe|||c|+| +ud deep po|||ou
+ud |uc|p|eu| |u.o|u||ou |º +||e+d, +pp+|eu| |o| ||e ºupe|||c|+| corp+||reu|. |o|e +u +dd|||ou+| ºr+|| |er+uç|or+
o| |u|+uc, ou ||e |e|| /,çor+||c |eç|ou. 8. B, ||e ||||| ,e+| ||e |er+uç|or+ ou ||e uoºe |+º +|roº| d|º+ppe+|ed
+ud ºo |+º ||e |eº|ou ou ||e /,çor+||c |eç|ou, ||+|, |oWe.e|, |+º |e|| + ºr+|| ºc+|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 196
FICük£ 9-16 hemao¿ìoma oI ìoIaocy ne|e || |u.o|.eº + |+|çe ºeçreu| o| º||u. w|||e |u.o|u||ou |º +||e+d,
+pp+|eu| ou ||e |o|e|e+d, ||e |eº|ou ou ||e uppe| e,e||d +ud ||e red|+| c+u||uº |º |rp+|||uç p|ope| |uuc||ou o|
||e ||d, +ud |||º |ud|c+|eº ||+| .|º|ou r|ç|| |e |rp+||ed |u ||e |u|u|e. |u |||º p+||eu|, ||e+|reu| W+º |ud|c+|ed.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 19T
FICük£ 9-1T hemao¿ìoma oI ìoIaocy, deep |esìoo I|e|e |º + |u||e|, r+ºº |u ||e ºu|cu||º +ººoc|+|ed
W||| + ºupe|||c|+| (|ed) po|||ou. I|eºe |eº|ouº |+|d|, |eç|eºº. I|e |er+uç|or+ W+º |ero.ed |, ºu|çe|,.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 198
FICük£ 9-18 Fyo¿eoìc
¿raou|oma â. I||º |º + ºo|||+|,
e|oded .+ºcu|+| uodu|e ||+| ||eedº
ºpou|+ueouº|, o| +||e| r|uo| ||+ur+.
I|e |eº|ouº uºu+||, |+.e + ºroo||
ºu||+ce, W||| o| W|||ou| c|uº|º, W|||
o| W|||ou| e|oº|ou. 8. 0u p+|rº
+ud ºo|eº ||e, |+.e + |,p|c+| co||+|
o| |||c|eued º||+|ur co|ueur +| ||e
|+ºe. I||º co||+| c+u |eº| |e ºeeu
W|eu .|eWed ||or ||e º|de, +º |º ||e
c+ºe |e|e.
â
8
|,oçeu|c ç|+uu|or+ |º + |+p|d|, de.e|op|uç .+º·
cu|+| |eº|ou uºu+||, |o||oW|uç r|uo| ||+ur+.
I||º |º + .e|, corrou ºo|||+|, e|oded .+ºcu|+|
uodu|e ||+| ||eedº ºpou|+ueouº|, o| +||e| r|uo|
||+ur+. I|e |eº|ou |+º + ºroo|| ºu||+ce, W|||
o| W|||ou| c|uº|º, W||| o| W|||ou| e|oº|ou (||ç.
9·¹3 1 ). || +ppe+|º +º + |||ç|| |ed, duº|, |ed,
.|o|+ceouº, o| ||oWu·||+c| p+pu|e W||| + co||+|
o| |,pe|p|+º||c ep|de|r|º +| ||e |+ºe (||ç. 9·¹3 3 )
+ud occu|º ou ||e ||uçe|º, ||pº, rou||, ||uu|, +ud
|oeº.
n|º|op+||o|oç|c+||, ||e|e +|e |o|u|+| +çç|eç+|eº o|
p|o|||e|+||uç c+p|||+||eº W||| eder+ +ud uure|ouº
ueu||op|||º. I|uº, p,oçeu|c ç|+uu|or+ |º ue|||e|
p,oçeu|c (+ººoc|+|ed W||| |+c|e||+| |u|ec||ou o|
||e º||u) uo| + ç|+uu|or+.
I|e+|reu| |º ºu|ç|c+| e\c|º|ou o| cu|e||+çe W|||
e|ec||odeº|cc+||ou +| ||e |+ºe.
I|e |rpo||+uce o| p,oçeu|c ç|+uu|or+ |º
||+| || c+u |e r|º|+|eu |o| +re|+uo||c uodu|+|
re|+uor+, +ud .|ce .e|º+.
F¥0C£NIC CkANüI0MA |C|·9 . o3o.¹

|C|·¹0 . |93.0
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 199
FICük£ 9-19 C|omus tumor â. I||º |º +u e\qu|º||e|, p+|u|u| ºu|uuçu+| uodu|e o| |edd|º| co|o|, p+|u |ecoreº
p+|o\,ºr+| upou e\poºu|e |o co|d. 8. C|oruº |uro| ou ||e p+|r o| + ¹o·,e+|·o|d |o,.
â 8
I||º |º + |uro| o| ||e ç|oruº |od,. I|e ¸|¤¤±º
|¤!, |º +u +u+|or|c +ud |uuc||ou+| uu|| cor·
poºed o| ºpec|+||/ed ºroo|| ruºc|e, ||e ¸|¤¤±º
:-||º ||+| ºu||ouud |||u·W+||ed eudo||e||+| ºp+ceº,
|||º +u+|or|c uu|| |uuc||ouº +º +u +||e||o.euouº
º|uu| ||u||uç +||e||o|eº +ud .euu|eº. I|e ç|oruº
ce||º ºu||ouud ||e u+||oW |ureu o| ||e 'ucque|·
no,e| c+u+| ||+| ||+uc|eº ||or ||e +||e||o|e +ud
|e+dº |o ||e co||ec||uç .euu|e ºeçreu| ||+| +c|º
+º + |eºe|.o||. C|oruº |od|eº +|e p|eºeu| ou ||e
p+dº +ud u+|| |edº o| ||e ||uçe|º +ud |oeº +ud
+|ºo ou ||e .o|+| +ºpec| o| |+udº +ud |ee|, |u ||e
º||u o| ||e e+|º, +ud |u ||e ceu|e| o| ||e |+ce.
I|e ç|oruº |uro| p|eºeu|º +º +u e\qu|º||e|, |eu·
de| ºu|uuçu+| o| ºu|cu|+ueouº p+pu|e o| uodu|e.
C|oruº |uro|º +|e c|+|+c|e||/ed |, p+|o\,ºr+|
p+|u|u| +||+c|º, eºpec|+||, e||c||ed |, e\poºu|e |o
co|d. I|e, +|e roº| o||eu p|eºeu| +º ºo|||+|, ºu|·
uuçu+| |uro|º (||ç. 9·¹9 1 ) |u| r+, |+|e|, occu|
+º ru|||p|e p+pu|eº o| uodu|eº. I|eºe +|e uo|ed,
eºpec|+||, |u c|||d|eu, +º d|ºc|e|e p+pu|eº o|
ºore||reº p|+queº +u,W|e|e ou ||e º||u ºu||+ce
(||ç. 9·¹9 3 ).
I|e|+p, |º |, e\c|º|ou.
CI0MüS IüM0k |C|·9 . 223.0

|C|·¹0 . \31¹¹/0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 200
FICük£ 9-20 Ao¿ìosarcoma â. E+||, |eº|ouº +ppe+| +º duº|, e|,||er+|ouº r+cu|eº. 8. \o|e +d.+uced
|eº|ouº +|e |ed |o ||+c| p+pu|eº +ud uodu|eº ||+| ||eed e+º||,. C. Ad.+uced +uç|oº+|cor+ W||| ||eed|uç pu|p|e |o
||+c| uodu|eº, u|ce|+||ou, +ud coucor||+u| eder+.
â C
8
I||º |º + |+|e, ||ç||, r+||çu+u| p|o|||e|+||ou o|
eudo||e||+| ce||º r+u||eº||uç +º pu|pu||c r+cu|eº
(||ç. 9·20 1 ) +ud/o| p+pu|eº +ud uodu|eº o|
|||ç|| |ed o| .|o|+ceouº +ud e.eu ||+c| co|o|
(||ç. 9·20 3 ). |odu|eº +|e ºo||d, ||eed e+º||,, +ud
u|ce|+|e (||ç. 9·20 C ).
I|e, occu| |u uo|r+| º||u, uºu+||, ou ||e ºc+|p
+ud uppe| |o|e|e+d o| |u |oc+||/ed |,rp|eder+,
|o| |uº|+uce |u poº|r+º|ec|or, |,rp|eder+
('|±c·|·í·-.-º º,¤!·¤¤- ) o| poº||||+d|+||ou
|,rp|eder+ (||ç. 9·203).
n|º|o|oç|c+||,. c|+uue|º ||ued |, p|eoro|p||c
eudo||e||+| ce||º W||| + ||ç| uur|e| o| r||oºeº.
I|e+|reu| |º |, ºu|çe|, +ud/o| c|ero||e|+p,
(||poºor+| do\o|u||c|u). I|e 5·,e+| ºu|.|.+| |º juº|
+|o.e ¹0°.
*Auç|oº+|cor+, +|||ouç| uo| + |e|çu ueop|+ºr, |º
d|ºcuººed |e|e |ec+uºe || |||º W||| o||e| .+ºcu|+| |uro|º.
ANCI0SAkC0MA |C|·¹0 . \9¹20/!
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 201
I|eºe +|e r+||o|r+||ouº ||+| do uo| uude|ço
ºpou|+ueouº |u.o|u||ou.
Cc¤·||c·, ¤c||¤·¤c|·¤¤º (C\º) (e.ç., ue.uº ||+r·
reuº, o| po||·W|ue º|+|u, +cco|d|uç |o ||e o|d
uoreuc|+|u|e), |,¤¤|c|·: ¤c||¤·¤c|·¤¤ :c¤·|·
|c·,·|,¤¤|c|·: ¤c||¤·¤c|·¤¤ (C|\), .-¤¤±º
¤c||¤·¤c|·¤¤ (\\), +ud c·|-··¤.-¤¤±º ¤c||¤··
¤c|·¤¤ (A\\) +|e d|º||uçu|º|ed.
n|º|o|oç|c+||, ||e, couº|º| o| eu|+|çed, |o||uouº
.eººe|º o| .+||ouº |,peº.
0u|, ||e roº| corrou +ud |rpo||+u| +|e |e|uç
de+|| W||| |e|e.
vASCüIAk MAIF0kMAII0NS
CAFIIIAk¥ MAIF0kMAII0NS
Skìo Iesìoos These aie maculai (Fig. 9-21)
with vaiying hues of pink to puiple. Laige
lesions follow a deimatomal distiibution and
aie usually unilateial (85%) though not always.
Most commonly involve the face wheie the CM
occuis in the distiibution of the tiigeminal
neive (Fig. 9-21), usually the supeiioi and
middle bianches; mucosal involvement of
conjunctiva and mouth may occui. CM may
also involve othei sites. With incieasing age
of the patient, papules oi iubbeiy nodules
(Fig. 9-22) often develop, leading to significant
disfiguiement.
C|ìoìca| varìaot
Ne·us [|ammeus nut|ae (°stoik bite," eiythema
nuchae, salmon patch) occuis in appioximately
one-thiid of infants on the nape of the neck and
tends to iegiess spontaneously. Similai lesions
may occui on eyelids and glabella. It is not ie-
ally a CM but iathei a tiansitoiy vasodilatation
phenomenon.
hISI0FAIh0I0C¥
Reveals ectasia of capillaiies and no piolifeia-
tion of endothelial cells. GLUT-1 immunoieac-
tivity is negative.
FICük£ 9-21 Fort-wìoe staìo '|+|p|, r+|ç|u+|ed,
po||·W|ue |ed r+cu|e occu|||uç |u + d|º||||u||ou o| ||e
ºecoud ||+uc| o| ||e |||çer|u+| ue|.e |u + c|||d.
A po||·W|ue º|+|u (|w') |º +u |||eçu|+||, º|+ped,
|ed o| .|o|+ceouº, r+cu|+| C\ ||+| |º p|eºeu| +|
||||| +ud ue.e| d|º+ppe+|º ºpou|+ueouº|,.
|| |º corrou (0.!° o| ueW|o|uº), ||e r+||o|r+·
||ou |º uºu+||, cou||ued |o ||e º||u.
\+, |e +ººoc|+|ed W||| .+ºcu|+| r+||o|r+||ouº
|u ||e e,e +ud |ep|oreu|uçeº ('|u|çe·we|e|
º,ud|ore).
',¤¤¤,¤ . |e.uº ||+rreuº.
F0kI-WIN£ SIAIN |C|·9 . 151.!2

|C|·¹0 . 032.5
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 202
C0ükS£ AN0 Fk0CN0SIS
PWSs aie CMs that do not iegiess spontane-
ously. The aiea of involvement tends to in-
ciease in piopoition to the size of the child. In
adulthood, PWSs usually become iaised with
papulai and nodulai aieas and aie the cause of
significant piogiessive cosmetic disfiguiement
(Fig. 9-22).
MANAC£M£NI
Duiing the maculai phase, PWS can be coveied
with makeup. Tieatment with tunable dye oi
coppei vapoi laseis is highly effective.
S¥N0k0MIC CM
Sìurge-Ve|er synJrome (SWS) is the association
of PWS in the tiigeminal distiibution with vas-
culai malfoimations in the eye and leptomenin-
ges and supeificial calcifications of the biain.
SWS may be associated with contialateial hemi-
paiesis, musculai hemiatiophy, epilepsy, and
mental ietaidation; glaucoma and oculai palsy
may occui. Skull x-iays show chaiacteiistic calci-
fications of vasculai malfoimations oi localized
lineai calcification along ceiebial convolutions.
CT scan should be done. It should, howevei, be
noted that PWS with tiigeminal distiibution
is common and does not necessaiily indicate
the piesence of SWS. K|í¡¡e|-Trénaunay-Ve|er
synJrome may have an associated PWS oveily-
ing the deepei vasculai malfoimation of soft
tissue and bone. PVS on ì|e míJ|íne |at| may
be associated with an undeilying aiteiiovenous
malfoimation of the spinal coid.
FICük£ 9-22 Fort-wìoe staìo w||| |uc|e+º|uç
+çe, ||e co|o| deepeuº +ud p+pu|+| +ud uodu|+| .+ºcu|+|
|eº|ouº de.e|op W||||u ||e p|e.|ouº|, r+cu|+| |eº|ou,
c+uº|uç p|oç|eºº|.e|, |uc|e+º|uç d|º||çu|ereu|.
'p|de| +uç|or+ |º + .e|, corrou |ed |oc+| |e|·
+uç|ec|+||c ue|Wo|| o| d||+|ed c+p|||+||eº |+d|+||uç
||or + ceu||+| +||e||o|e (puuc|ur) (||ç. 9·2! 1 ).
I|e ceu||+| p+pu|+| puuc|ur |º ||e º||e o| ||e
|eed|uç +||e||o|e W||| r+cu|+| |+d|+||uç |e|+uç|ec·
|+||c .eººe|º. up |o ¹.5 cr |u d|+re|e|. uºu+||,
ºo|||+|,.
0u d|+ºcop,, ||e |+d|+||uç |e|+uç|ec|+º|+ ||+uc|eº
+ud ||e ceu||+| +||e||o|e r+, pu|º+|e.
\oº| corrou|, occu|º ou ||e |+ce, |o|e+|rº, +ud
|+udº.
|| ||equeu||, occu|º |u uo|r+| pe|ºouº +ud |º
ro|e corrou |u |er+|eº, occu|º |u c|||d|eu.
|| r+, |e +ººoc|+|ed W||| |,pe|eº||oçeu|c º|+|eº,
ºuc| +º p|eçu+uc, (oue o| ro|e |u |Wo·||||dº o|
p|eçu+u| Woreu), o| occu|º |u p+||eu|º |ece|.·
|uç eº||oçeu ||e|+p,, e.ç., o|+| cou||+cep||.eº,
o| |u ||oºe W||| |ep+|oce||u|+| d|ºe+ºe ºuc| +º
ºu|+cu|e +ud c||ou|c .||+| |ep+||||º +ud +|co|o||c
c||||oº|º (||ç. 9·2! 3 ).
'p|de| +uç|or+ +||º|uç |u c|||d|ood +ud p|eç·
u+uc, r+, |eç|eºº ºpou|+ueouº|,.
I|e |eº|ou r+, |e cou|uºed W||| |-·-!·|c·, |-¤·
¤··|c¸·: |-|c¤¸·-:|cº·c c|c··c·|-|c¤¸·-:|cº·c o|
|-|c¤¸·-:|cº·c |u º,º|er|c ºc|e|ode|r+.
|eº|ouº r+, |e ||e+|ed e+º||, W||| e|ec||o· o| |+ºe|
ºu|çe|,.
',¤¤¤,¤º. |e.uº +|+ueuº, ºp|de| ue.uº, +||e||+|
ºp|de|, ºp|de| |e|+uç|ec|+º|+, .+ºcu|+| ºp|de|.
SFI0£k ANCI0MA |C|·9 . ++3.¹

|C|·¹0 . ¹13.¹
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 203
â
FICük£ 9-23 Spìder oevus â. IWo ºr+|| p+pu|eº ||or W||c| |e|+uç|ec|+º|+º |+d|+|e. upou corp|eºº|ou ||e
|eº|ou ||+uc|eº corp|e|e|,. 8. 'p|de| ue.| ou ||e c|eº| o| + p+||eu| W||| c||||oº|º.
8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 204
FICük£ 9-24 veoous |ake â. 0u ||e c|ee| o| + 10·,e+|·o|d r+|e. I|e |eº|ou W+º +|roº| ||+c| +ud |ec+re
+ r+||e| o| couce|u |o ||e p+||eu|, W|o |e+|ed |e r|ç|| |+.e re|+uor+. noWe.e|, || ||+uc|ed corp|e|e|, +||e|
corp|eºº|ou. 8. \euouº |+|e ou ||e c|ee| o| +u 32·,e+|·o|d |er+|e. I||º ||u|º|·||+c| uodu|e ||+uc|ed
corp|e|e|, +||e| corp|eºº|ou.
â 8
A .euouº |+|e |º + d+|| ||ue |o .|o|+ceouº,
+º,rp|or+||c, ºo|| p+pu|e |eºu|||uç ||or + d||+|ed
.euu|e, occu|||uç ou ||e |+ce, ||pº, +ud e+|º o|
p+||eu|º >50 ,e+|º o| +çe (||ç. 9·2+ 1 , 3 ).
I|e e||o|oç, |º uu|uoWu, |u| || |+º |eeu |e|+|ed
|o ºo|+| e\poºu|e.
I|eºe |eº|ouº +|e |eW |u uur|e| +ud |er+|u |o|
,e+|º. I|e |eº|ou |eºu||º ||or + d||+|ed c+.||, ||ued
W||| + º|uç|e |+,e| o| ||+||eued eudo||e||+| ce||º
+ud + |||u W+|| o| ||||ouº ||ººue ||||ed W||| |ed
||ood ce||º.
|ue |o ||º d+|| ||ue o| ºore||reº e.eu ||+c|
co|o|, ||e |eº|ou r+, |e cou|uºed W||| uodu|+|
re|+uor+ o| p,oçeu|c ç|+uu|or+.
I|e |eº|ou c+u |e p+|||+||, corp|eººed +ud ||ç||·
eued up |, d|+ºcop,, +ud ||e uºe o| de|roºcop,
pe|r||º ||º e+º, d|+çuoº|º +º + .+ºcu|+| |eº|ou.
\+u+çereu| |º |o| coºre||c |e+ºouº +ud c+u |e
+ccorp||º|ed W||| e|ec||oºu|çe|,, |+ºe|, o|, |+|e|,,
W||| ºu|ç|c+| e\c|º|ou.
v£N0üS IAk£ |C|·9 . 523.5

|C|·¹0 . K¹!.0
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 205
FICük£ 9-25 Cherry ao¿ìomas I|eºe |||ç|| |ed, .|o|+ceouº o| e.eu ||+c| |eº|ouº +ppe+| p|oç|eºº|.e|, ou
||e ||uu| W||| +d.+uc|uç +çe.
C|e||, +uç|or+º +|e e\ceed|uç|, corrou,
+º,rp|or+||c, |||ç|| |ed |o .|o|+ceouº o| e.eu
||+c|, dored .+ºcu|+| |eº|ouº ( ! rr) (||ç.
9·25) o| occu|||uç +º r,||+dº o| ||u, |ed p+pu|+|
ºpo|º º|ru|+||uç pe|ec||+e.
I|e, +|e |ouud p||uc|p+||, ou ||e ||uu|. I|e |e·
º|ouº +ppe+| |||º| +| +|ou| +çe !0 +ud |uc|e+ºe |u
uur|e| o.e| ||e ,e+|º.
I|e|e +|e |+|d|, +u, e|de||, peop|e W|o do uo|
|+.e +| |e+º| + |eW |eº|ouº.
I|e ||º|o|oç, couº|º|º o| uure|ouº rode|+|e|,
d||+|ed c+p|||+||eº ||ued |, ||+||eued eudo||e||+|
ce||º, º||or+ |º eder+|ouº W||| |oroçeu|/+||ou
o| co||+çeu.
I|e, +|e o| uo couºequeuce o||e| ||+u ||e||
coºre||c +ppe+|+uce. \+u+çereu| |º e|ec||o· o|
|+ºe| co+çu|+||ou || |ud|c+|ed coºre||c+||,. C|,o·
ºu|çe|, |º uo| e||ec||.e.
',¤¤¤,¤º. C+rp|e|| de \o|ç+u ºpo|º, ºeu||e
(|er)+uç|or+.
Ch£kk¥ ANCI0MA |C|·9 . 223.0

|C|·¹0 . ¹13.3
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 206
FICük£ 9-26 Ao¿ìokeratoma: so|ìtary I||º ||+c|, |||r |eº|ou W||| + pe|||ed ºu||+ce |rred|+|e|, ºp+||º
||e ºuºp|c|ou o| ºupe|||c|+| ºp|e+d|uç re|+uor+. || |º uoucorp|eºº|||e, |u| de|roºcop, |e.e+|º ||e |,p|c+| |+cu·
u+e o| |||or|oºed .+ºcu|+| ºp+ceº. |oue||e|eºº, ºuc| |eº|ouº º|ou|d |e e\c|ºed.
I|e |e|r c¤¸·¤ ('||ood .eººe|¨) |-·c|¤¤c Wou|d
|rp|, + .+ºcu|+| |uro| W||| |e|+|o||c e|ereu|º.
Bu|, |u |+c|, c+p|||+||eº +ud poº|c+p|||+|, .euu|eº
+|e p+c|ed |u|o ||e p+p|||+|, |od, juº| |eue+||
+ud |u|ç|uç |u|o ||e ep|de|r|º, |e+d|uç |o |,pe|·
|e|+|oº|º. I||º +ud ||e |+c| ||+| ||e |ur|u+ +|e
uºu+||, +| |e+º| p+|||+||, |||or|oºed |rp+|| + |||r
couº|º|euc, |o ||e |eº|ouº.
Auç|o|e|+|or+º +|e d+|| .|o|+ceouº |o ||+c|,
o||eu |e|+|o||c p+pu|eº o| ºr+|| p|+queº ||+| +|e
|+|d upou p+|p+||ou +ud c+uuo| |e corp|eººed
|, d|+ºcop, (||ç. 9·2o).
Auç|o|e|+|or+ c+u +ppe+| +º + ºo|||+|, |eº|ou
(º¤|·|c·, c¤¸·¤|-·c|¤¤c ), +ud ||eu ||e roº|
|rpo||+u| d|||e|eu||+| d|+çuoº|º |º + ºr+|| uodu|+|
o| ºupe|||c|+| ºp|e+d|uç re|+uor+ (||ç. 9·2o).
I|e roº| corrou |º c¤¸·¤|-·c|¤¤c ¤| |¤·!,:- ,
|||º d|ºe+ºe |u.o|.eº ||e ºc|o|ur +ud .u|.+, ||e
|eº|ouº +|e ru|||p|e p+pu|eº ( + rr) ||+| +|e
d+|| |ed |u co|o| +ud p|eºeu| |u qu||e |+|çe uur·
|e|º (||ç. 9·21).
1¤¸·¤|-·c|¤¤c ¤| !·|-||· corp||ºeº p|u| |o
d+|| |ed +ud e.eu ||+c| p+pu|eº ||+| occu| ou
||e e||oWº, |ueeº, +ud do|º+ o| ||e |+udº. I||º
+u|oºor+| dor|u+u| d|ºe+ºe |º |+|e +ud occu|º |u
,ouuç |er+|eº.
1¤¸·¤|-·c|¤¤c :¤·¤¤··º !·||±º±¤ ( |c|·, !·º-cº-),
+u /·||u|ed |eceºº|.e d|ºe+ºe, |º +u |u|o|u e||o| o|
re|+|o||ºr |u W||c| ||e|e |º + de||c|euc, o|
·ç+|+c|oº|d+ºe A |e+d|uç |o +u +ccuru|+||ou o|
ueu||+| ç|,coºp||uço||p|d ce|+r|de ||||e\oº|de |u
eudo||e||+| ce||º, ||||oc,|eº, +ud pe||c,|eº |u ||e
de|r|º, |e+||, ||due,º, +ud +u|ouor|c ue|.ouº
º,º|er. |eº|ouº +|e uure|ouº d+|| |ed, puuc|+|e,
+ud ||u, (·¹ rr) (||ç. 9·23), |oc+|ed ou ||e
|oWe| |+|| o| ||e |od,. |oWe| +|doreu, çeu||+||+,
+ud |u||oc|º, +|||ouç| |eº|ouº r+, +|ºo occu| ou
||e ||pº. I|e |oro/,çouº r+|eº |+.e uo| ou|, ||e
º||u |eº|ouº |u| +|ºo º,rp|orº |e|+|ed |o |u.o|.e·
reu| o| o||e| o|ç+u º,º|erº. +c|op+|eº||eº|+º,
e\c|uc|+||uç p+|u, ||+uº|eu| |ºc|er|c +||+c|º, +ud
r,oc+|d|+| |u|+|c||ou. ne|e|o/,çouº |er+|eº r+,
|+.e co|ue+| op+c|||eº. |+||, d|ºe+ºe |º |+|e.
ANCI0k£kAI0MA |C|·9 . ++3.9

|C|·¹0 . \9¹+¹/0
*
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 20T
FICük£ 9-2T Ao¿ìokeratoma oI
Fordyce kedd|º|, .|o|+ceouº +ud ||+c|
p+pu|eº ou ||e ºc|o|ur. I|e, ||+uc| upou
d|+ºcop, +ud |||º .e||||eº ||e d|+çuoº|º.
|o|e. I||or|oºed +uç|o|e|+|or+º do uo|
||+uc|.
FICük£ 9-28 Ao¿ìokeratoma
corporìs dìIIusum (Fabry dìsease)
|ure|ouº |ed, puuc|+|e |eº|ouº ou
||e |oWe| ||+u|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 208
I¥MFhAIIC MAIF0kMAII0N (IM)
FICük£ 9-29 Iymphatìc ma|Iormatìoo (|ymphao¿ìoma) ||oç·ºp+Wu|||e cou||ueu| ç|ouped '.eº|c|eº¨
||||ed W||| + ºe|oº+uçu|ueouº ||u|d.
I|e |e|r |,¤¤|c|·: ¤c||¤·¤c|·¤¤ |º ||e |e|r|·
uo|oç, |o| W|+| W+º |o|re||, c+||ed '|,rp|+uç|·
or+.¨
I|eºe |,p|c+| |eº|ouº corp||ºe ru|||p|e, ç|ouped,
ºr+|| r+c|oºcop|c .eº|c|eº ||||ed W||| c|e+| o|
ºe|oº+uçu|ueouº ||u|d ('||oç·ºp+Wu¨) (||ç. 9·
29). noWe.e|, ||eºe +|e uo| ||ue .eº|c|eº |u|
r|c|oc,º||c |eº|ouº (|,rp|+uç|or+) +º oppoºed
|o + r+c|oc,º||c |eº|ou (c,º||c |,ç|or+), W||c|
|º |oc+|ed deep |u ||e de|r|º +ud ºu|cu||º +ud
+ppe+|º +º + |+|çe ºo|| ºu|cu|+ueouº |uro| o||eu
d|º|o|||uç ||e |+ce o| +u e\||er||,.
I|e r|c|oc,º||c |\ |º p|eºeu| +| ||||| o| +ppe+|º
|u |u|+uc, o| c|||d|ood. || r+, d|º+ppe+| ºpou·
|+ueouº|,, |u| |||º |º e\||ere|, |+|e. B+c|e||+|
|u|ec||ou r+, occu|.
|\ r+, occu| +º +u |ºo|+|ed ºo|||+|, |eº|ou, +º
|u ||ç. 9·29, o| co.e| |+|çe +|e+º (up |o ¹0  20
cr), || r+, |e +ººoc|+|ed W||| + c+p|||+|, .euouº
|,rp|+||c (C\|) r+||o|r+||ou.
I|e |eº|ou c+u |e e\c|ºed, || |e+º|||e, o| ||e+|ed
W||| ºc|e|o||e|+p,.
I¥MFhANCI0MA |C|·9 . 223.¹

|C|·¹0 . |¹3.¹·\9¹10/0
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 209
vAkIANIS
vascu|ar hamartomas CVLs with deep soft
tissue involvement and iesultant swelling oi dif-
fuse enlaigement of an extiemity. May involve
skeletal muscle with muscle atiophy. Cutaneous
changes include dilated toituous veins and aite-
iiovenous fistulas.
C\\º +|e deep .+ºcu|+| r+||o|r+||ouº c|+|+c|e|·
|/ed |, ºo||, corp|eºº|||e deep·||ººue ºWe|||uç.
|eº|ouº +|e uo| +pp+|eu| +| ||||| |u| |ecore ºo
du||uç c|||d|ood.
I|e, r+u||eº| +º ºo|| ||ººue ºWe|||uç, dore·
º|+ped o| ru|||uodu|+| (||ç. 9·!0), +ud +|e
º|oW·||oW |eº|ouº. w|eu .+ºcu|+| r+||o|r+||ou
e\|eudº |o ||e ep|de|r|º, ||e ºu||+ce r+, |e .e|·
|ucouº. I|e |o|de|º +|e poo||, de||ued, +ud ||e|e
|º couº|de|+||e .+||+||ou |u º|/e. 0||eu, C\\º +|e
uo|r+| º||u co|o|, W||| ||e uodu|+| po|||ou ||ue
|o pu|p|e. I|e, +|e e+º||, corp|eººed +ud ||||
p|orp||, W|eu p|eººu|e |º |e|e+ºed. 'ore |,peº
r+, |e |eude|, +ud ||e, r+, |e +ººoc|+|ed W|||
C\º.
C\\º r+, |e corp||c+|ed |, u|ce|+||ou +ud
||eed|uç, ºc+|||uç, +ud ºecoud+|, |u|ec||ou, +ud,
W||| |+|çe |eº|ouº, |, ||ç|·ou|pu| |e+|| |+||u|e.
C\\º r+, |u|e||e|e W||| |ood |u|+|e o| ||e+|||uç
+ud, || |oc+|ed ou ||e e,e||dº o| |u ||e .|c|u||,
o| ||e e,eº, W||| o|º||uc| .|º|ou +ud r+, |e+d |o
|||udueºº.
I|e|e |º uo º+||º|+c|o|, ||e+|reu| e\cep| cor·
p|eºº|ou. |u |+|çe| |eº|ouº-|| o|ç+u |uuc||ou |º
corp|or|ºed-ºu|ç|c+| p|ocedu|eº +ud |u||+.+º·
cu|+| co+çu|+||ou º|ou|d |e pe||o|red. n|ç|·
doºe º,º|er|c ç|ucoco|||co|dº o| |||· r+, |e
e||ec||.e.
|C|·9 . 151.!2
CAFIIIAk¥[v£N0üS MAIF0kMAII0NS (CvMs)
k|ìppe|-Iréoauoay Syodrome A CVM oi
CVL malfoimation, slow-flow lesion. Local
oveigiowth of soft tissue and bone iesults in
enlaigement of an extiemity. Associated cu-
taneous changes include phlebectasia, nevus
flammeus-like cutaneous CM (Fig. 9-31), lym-
phatic hypoplasia, and lymphedema.
FICük£ 9-30 Capì||ary-veoous ma|Iormatìoo |u +u |u|+u|. I|e|e |º + ºo||, corp|eºº|||e, ||u|º|·|ed ||ººue
ºWe|||uç d|º|o|||uç ||e uppe| ||p +ud |oWe| e,e||d. || |º + º|oW·||oW |eº|ou |u| |equ||eº ||e|+peu||c |u|e|.eu||ou.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 210
8|ue kubber 8|eb Nevus A venous malfoi-
mation that is spontaneously painful and/oi
tendei. It is a compiessible, soft, blue swelling in
the deimis and subcutaneous tissue. Size ianges
fiom a few millimeteis to seveial centimeteis
(Fig. 9-32). The lesion may exhibit localized
hypeihidiosis ovei CVL malfoimations and
occuis, often multiply, on the tiunk and uppei
aims. Similai vasculai lesions can occui in the
gastiointestinal tiact and may be a souice of
hemoiihage.
MarIuccì Syodrome A slow-flow venous oi
lymphatic/venous malfoimation associated
with enchondiomas and manifested as haid
nodules on fingeis oi toes and as bony defoimi-
ties. Patients may develop chondiosaicoma.
Farkes-Weber Syodrome A fast-flow capillaiy
aiteiiovenous malfoimation (CAVM) oi CM,
with soft tissue and skeletal hypeitiophy.
FICük£ 9-31 Capì||ary-veoous ma|Iormatìoo |u +
!¹·,e+|·o|d Wor+u. I||º ue.uº ||+rreuº-|||e |eº|ou W+º
+ººoc|+|ed W||| p||e|ec|+º|+, |,rp|eder+, +ud +u eu|+|çed
||ç|| |oWe| e\||er||, (K||ppe|·I|eu+uu+, º,ud|ore).
FICük£ 9-32 8|ue rubber b|eb oevus A ºpou|+ueouº|, p+|u|u| +ud |eude| .euouº r+||o|r+||ou. I|e|e
+|e + uur|e| o| corp|eºº|||e ||u|º|·.|o|+ceouº p+pu|eº +ud uodu|eº ou ||e uppe| +|r.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 211
MISC£IIAN£0üS C¥SIS AN0 FS£ü00C¥SIS
FICük£ 9-33 £pìdermoìd cyst â. A |ouuded uodu|e W||||u ||e de|r|º. |o| +|W+,º |º ||e|e +u opeu|uç
|||ouç| W||c| c+ºeouº |e|+||uouº r+|e||+| c+u |e e\p|eººed. 8. kup|u|ed ep|de|ro|d c,º|. I|eºe |u||+rr+|o|,
|eº|ouº +|e o||eu r|ºd|+çuoºed +º |e|uç |u|ec|ed.
â 8
Au ep|de|ro|d c,º| |º ||e roº| corrou cu|+ue·
ouº c,º|, de||.ed ||or ep|de|r|º o| ||e ep|||e·
||ur o| ||e |+|| |o|||c|e, +ud |º |o|red |, c,º||c
euc|oºu|e o| ep|||e||ur W||||u ||e de|r|º ||+|
|ecoreº ||||ed W||| |e|+||u +ud ||p|d·||c| de|||º.
|| occu|º |u ,ouuç |o r|dd|e·+çed +du||º ou ||e
|+ce, uec|, uppe| ||uu|, +ud ºc|o|ur.
I|e |eº|ou, W||c| |º uºu+||, ºo|||+|, |u| r+, |e
ru|||p|e, |º + de|r+|·|o·ºu|cu|+ueouº uodu|e,
0.5-5 cr, W||c| o||eu couuec|º W||| ||e ºu||+ce
|, |e|+||u·||||ed po|eº (||ç. 9·!! 1 ).
I|e c,º| |+º +u ep|de|r+|·|||e W+|| (º||+||||ed
ºqu+rouº ep|||e||ur W||| We||·|o|red ç|+uu|+|
|+,e|), ||e cou|eu| o| ||e c,º| |º |e|+||u+ceouº
r+|e||+|-c|e+r·co|o|ed W||| + p+º|, couº|º|euc,
+ud ||e odo| o| |+uc|d c|eeºe. 'c|o|+| |eº|ouº r+,
c+|c||,.
I|e c,º| W+|| |º |e|+||.e|, |||u. |o||oW|uç |up|u|e
o| ||e W+||, ||e |||||+||uç c,º| cou|eu|º |u|||+|e
+u |u||+rr+|o|, |e+c||ou, eu|+|ç|uç ||e |eº|ou
r+u,|o|d, ||e |eº|ou |º uoW +ººoc|+|ed W||| +
ç|e+| de+| o| p+|u. kup|u|ed c,º|º (||ç. 9·!! 3 ) +|e
o||eu r|ºd|+çuoºed +º |e|uç |u|ec|ed |+||e| ||+u
|up|u|ed.
',¤¤¤,¤º weu, ºe|+ceouº c,º|, |u|uud||u|+|
c,º|, ep|de|r+| c,º|.
£FI0£kM0I0 C¥SI |C|·9 . 10o.2

|C|·¹0 . |12.0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 212
FICük£ 9-34 Irìchì|emma| cyst A |||r, dore·
º|+ped uodu|e ou ||e ºc+|p. ||eººu|e |, ||e c,º|
|+º c+uºed +||op|, o| |+|| |u||º +ud || ||uº +ppe+|º
W|||ou| |+||º.
FICük£ 9-35 £pìderma| ìoc|usìoo cyst A
ºr+|| de|r+| uodu|e ou ||e |uee +| ||e º||e o| ||e
|+ce|+||ou.
A |||c|||err+| c,º| |º ||e ºecoud roº| corrou
|,pe o| cu|+ueouº c,º| +ud |º ºeeu roº| o||eu |u
r|dd|e +çe, ro|e ||equeu||, |u |er+|eº. || |º o||eu
|+r|||+| +ud occu|º ||equeu||, +º ru|||p|e |eº|ouº.
I|eºe +|e ºroo||, |||r, dore·º|+ped, 0.5· |o
5·cr uodu|eº o| |uro|º, ||e, |+c| ||e ceu||+|
puuc|ur ºeeu |u ep|de|ro|d c,º|º. I|e, +|e uo|
couuec|ed |o ||e ep|de|r|º.
0.e| 90° occu| ou ||e ºc+|p, +ud ||e o.e||,|uç
ºc+|p |+|| |º uºu+||, uo|r+| |u| r+, |e |||uued ||
||e c,º| |º |+|çe (||ç. 9·!+).
 I|e c,º| W+|| |º uºu+||, |||c|, +ud ||e c,º| c+u |e
|ero.ed |u|+c|. I|e W+|| |º + º||+||||ed ºqu+rouº
ep|||e||ur W||| + p+||º+ded ou|e| |+,e| |eºer|||uç
||+| o| ||e ou|e| |oo| º|e+|| o| |+|| |o|||c|eº. I|e
|uue| |+,e| |º co||uç+|ed W|||ou| + ç|+uu|+| |+,e|.
I|e c,º| cou|+|uº |e|+||u-.e|, deuºe, |oroçe·
ueouº, || |º o||eu c+|c|||ed, W||| c|o|eº|e|o| c|e||º.
|| c,º| |up|u|eº, || r+, |e |u||+red +ud .e|,
p+|u|u|.
',¤¤¤,¤º |||+| c,º|, |º||ruº c+|+çeu c,º|. 1··
:|c·: |e|rº. weu, ºe|+ceouº c,º|.
IkIChII£MMAI C¥SI |C|·9 . 10o.2

|C|·¹0 . |12.0
Au ep|de|r+| |uc|uº|ou c,º| occu|º ºecoud+|,
|o ||+ur+||c |rp|+u|+||ou o| ep|de|r|º |u|o ||e
de|r|º. I|+ur+||c+||, ç|+||ed ep|de|r|º ç|oWº |u
||e de|r|º, W||| +ccuru|+||ou o| |e|+||u W||||u
||e c,º| c+.||,, euc|oºed |u + º||+||||ed ºqu+rouº
ep|||e||ur W||| + We||·|o|red ç|+uu|+| |+,e|.
I|e |eº|ou +ppe+|º +º + de|r+| uodu|e (||ç. 9·!5)
+ud roº| corrou|, occu|º ou ||e p+|rº, ºo|eº,
+ud ||uçe|º.
|| º|ou|d |e e\c|ºed.
',¤¤¤,¤ . I|+ur+||c ep|de|ro|d c,º|.
£FI0£kMAI INCIüSI0N C¥SI
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 213
FICük£ 9-36 Mì|ìum â. A ºr+|| c|+||·W|||e
o| ,e||oW|º| p+pu|e ou ||e c|ee|, || c+u |e º|||
W||| + ºc+|pe|, |e|e+º|uç + |||||e |+|| o| |o|u, r+|e·
||+|. 8. A º||ç|||, |+|çe| |eº|ou ou ||e |oWe| ||d o|
+u A|||c+u Wor+u. C. \u|||p|e r|||+ ou ||e ||uu|
o| + c|||d W||| |e|ed||+|, d,º||op||c ep|de|ro|,º|º
|u||oº+ (ºee 'ec||ou o).
â
8
C
A r|||ur |º + ¹· |o 2·rr, ºupe|||c|+|, W|||e |o
,e||oW, |e|+||u·cou|+|u|uç ep|de|r+| c,º|, occu|·
||uç ru|||p|,, |oc+|ed ou ||e e,e||dº, c|ee|º, +ud
|o|e|e+d |u p||oºe|+ceouº |o|||c|eº (||ç. 9·!o 1, 3 ).
I|e |eº|ouº c+u occu| +| +u, +çe, e.eu |u |u|+u|º.
\|||+ +||ºe e|||e| de uo.o, eºpec|+||, +|ouud ||e
e,e, o| |u +ººoc|+||ou W||| .+||ouº de|r+|oºeº
W||| ºu|ep|de|r+| |u||+e o| .eº|c|eº (perp||ç·
o|d, po|p|,||+ cu|+ue+ |+|d+, |u||ouº ||c|eu p|+·
uuº, ep|de|ro|,º|º |u||oº+) (||ç. 9·!o C ) +ud º||u
||+ur+ (+||+º|ou, |u|uº, de|r+||+º|ou, |+d|+||ou
||e|+p,).
|uc|º|ou +ud e\p|eºº|ou o| cou|eu|º +|e ||e
re||od o| ||e+|reu|.
MIIIüM
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 214
FICük£ 9-3T 0ì¿ìta| myxoìd cyst â. I|e c,º| |+º |ed |o + !· |o +·rr ç|oo.e o| ||e u+|| p|+|e. 8. '|||||uç
|| W||| + ºc+|pe| +ud p|eººu|e |e|e+ºeº + çe|+||uouº .|ºcouº ||u|d.
8 â
A d|ç||+| r,\o|d c,º| |º + pºeudoc,º| occu|||uç
o.e| ||e d|º|+| |u|e|p|+|+uçe+| jo|u| +ud ||e |+ºe
o| ||e u+|| o| ||e ||uçe| (||ç. 9·!1 1 ) o| |oe, o||eu
+ººoc|+|ed W||| ne|e|deu'º (oº|eop|,||c) uode.
I|e |eº|ou occu|º |u o|de| p+||eu|º, uºu+||, >o0
,e+|º o| +çe.
|| |º uºu+||, + ºo|||+|, c,º|, |u||e|,, ||+uº|uceu|.
A c|e+| çe|+||uouº .|ºcouº ||u|d r+, |e e\||uded
(||ç. 9·!1 3 ).
w|eu ||e r,\o|d c,º| |º o.e| ||e u+|| r+|||\, +
u+|| p|+|e d,º||op|, occu|º |u ||e |o|r o| + ¹· |o
2·rr ç|oo.e ||+| e\|eudº |o ||e |euç|| o| ||e
u+|| (||çº.9·!1 1 , !!·¹2). ('ee 'ec||ou !!.)
\+||ouº re||odº o| r+u+çereu| |+.e |eeu
+d.oc+|ed, |uc|ud|uç ºu|ç|c+| e\c|º|ou, |uc|º|ou
+ud d|+|u+çe, |ujec||ou o| ºc|e|oº|uç r+|e||+|, +ud
|ujec||ou o| + |||+rc|uo|oue ºuºpeuº|ou. A º|rp|e
+ud roº| e||ec||.e re||od |º |o r+|e + ºr+||
|uc|º|ou, e\p|eºº ||e çe|+||uouº cou|eu|º, +ud uºe
+ |||r corp|eºº|ou |+ud+çe o.e| ||e |eº|ou o.e|
+ pe||od o| Wee|º.
',¤¤¤,¤º . \ucouº c,º|, º,uo.|+| c,º|, r,\o|d
pºeudoc,º|.
0ICIIAI M¥X0I0 C¥SI |C|·9 . 121.+¹
°
|C|·¹0 . \25.3
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 215
£FI0£MI0I0C¥
0oset Raiely befoie 30 yeais.
Sex Slightly moie common and moie exten-
sive involvement in males.
CIINICAI MANIF£SIAII0N
Evolve ovei months to yeais. Raiely piuiitic;
tendei if secondaiily infected.
Skìo Iesìoos Eur|y Small, 1- to 3-mm, baiely
elevated papule, latei a laigei plaque (Figs. 9-38
MISC£IIAN£0üS 8£NICN N£0FIASMS AN0 h¥F£kFIASIAS
FICük£ 9-38 Seborrheìc keratosìs, so|ìtary A º||ç|||, |+|ºed, |e|+|o||c, ||oWu, ||+| p|+que ou ||e /,ço·
r+||c |eç|ou |u +u o|de| |er+|e. I|e d|||e|eu||+| d|+çuoº|º |uc|udeº |eu||ço r+||çu+ +ud |eu||ço r+||çu+ re|+uor+.
I|e ºe|o|||e|c |e|+|oº|º |º ||e roº| corrou o|
||e |eu|çu ep|||e||+| |uro|º.
I|eºe |eº|ouº, W||c| +|e |e|ed||+|,, do uo| +ppe+|
uu||| +çe !0 +ud cou||uue |o occu| o.e| + |||e||re,
.+|,|uç |u e\|eu| ||or + |eW ºc+||e|ed |eº|ouº |o
|||e|+||, |uud|edº |u ºore .e|, e|de||, p+||eu|º.
|eº|ouº |+uçe ||or ºr+||, |+|e|, e|e.+|ed p+pu|eº
|o p|+queº W||| + W+||, ºu||+ce +ud + 'º|uc| ou¨
+ppe+|+uce.
|eº|ouº +|e |eu|çu +ud do uo| |equ||e ||e+|reu|
e\cep| |o| coºre||c |e+ºouº. I|e, c+u |ecore
|||||+|ed o| ||+ur+||/ed, W||| p+|u +ud ||eed|uç.
'CC º|ou|d |e |u|ed ou|.
',¤¤¤,¤ .-··±:c º-|¤··|¤·:c
S£80kkh£IC k£kAI0SIS |C|·9 .102.¹
°
|C|·¹0 . |32
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 216
and 9-39) with oi without pigment. The suiface
has a gieasy feel and often shows, with a hand
lens, fine stippling like the suiface of a thimble.
Elevation can be demonstiated by lightly fieezing
the lesion with LN2. Lesions such as lentigo
(benigna oi maligna) aie macules; SKs have a
shaiply maiginated elevated edge in compaiison.
Lute Plaque with waity suiface and °stuck on"
appeaiance (Fig. 9-40), °gieasy." With a hand
lens hoin cysts can often be seen; with deimos-
copy they can always be seen and aie diagnostic.
Size fiom 1 to 6 cm. Flat nodule. Biown, giay,
black, skin-coloied, iound oi oval (Figs. 9-39
and 9-40 À , B ).
DIstrIhutIvn Isolated lesion oi geneialized.
Face, tiunk (Fig. 9-41), uppei extiemities. In
daik skinned people, multiple, small black
lesions in the face aie called Jermaìosís ¡a¡u|osa
nígra (Fig. 9-39). SKs aie most dense in sun-
exposed site with deimatoheliosis. When nu-
meious and dense, SKs may become confluent.
In females, commonly occui in submammaiy
inteitiiginous skin.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Piolifeiation of mono-
moiphous keiatinocytes (with maiked papil-
lomatosis) and melanocytes, foimation of hoin
cysts. Some lesions can exhibit atypia of keiati-
nocytes, mimicking Bowen disease (SCCIS), flat
oi squamous cell caicinoma (SCC), and these
should be excised.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinically, the diagnosis is made easily. Cuiet-
tage may be helpful: seboiiheic keiatosis comes
off easily aftei slight fieezing and peimits his-
topathologic examination.
"Iao Macu|es" Eaily °flat" lesions may be con-
fused with solai lentigo oi spieading pigmented
actinic keiatosis (see Fig. 10-28).
Skìo-Co|ored[Iao[8|ack verrucous Fapu|es[
F|aques Laigei pigmented lesions aie easily
mistaken foi pigmented basal cell caicinoma
(BCC) oi malignant melanoma (only biopsy
will settle this, oi deimoscopy will be of assist-
ance); veiiuca vulgaiis may be similai in clini-
cal appeaiance, but thiombosed capillaiies aie
piesent in veiiucae.
C0ükS£ AN0 Fk0CN0SIS
Lesions develop with incieasing age; they aie
benign and do not become malignant.
MANAC£M£NI
Light electiocauteiy peimits the whole lesion
to be easily iubbed off. Then the base can
be lightly cauteiized to pievent iecuiience.
This, howevei, piecludes histopathologic veii-
fication of diagnosis and should be done only
by an expeiienced diagnostician. Ciyosuigeiy
with liquid nitiogen spiay woiks only in flat
lesions, and iecuiiences aie possibly moie
fiequent. The best appioach is cuiettage af-
tei slight fieezing with ciyospiay, which also
peimits histopathologic examination. In a
solid black lesion without hoin cysts, a punch
biopsy is mandatoiy to iule out malignant
melanoma; in this case a shave biopsy should
not be peifoimed as, in the case of melanoma,
it will not peimit evaluation of the level of
invasion.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 21T
FICük£ 9-39 Seborrheìc keratosìs (dermatosìs papu|osa oì¿ra) I||º couº|º|º o| + r,||+d o| ||u, ||+c|
|eº|ouº, ºore eu|+|ç|uç |o ro|e ||+u + ceu||re|e|. I||º |º ºeeu |u B|+c| A|||c+uº, A|||c+u Are||c+uº, +ud deep|,
p|çreu|ed 'ou|| E+º| Aº|+uº. I|e+|reu| |º + p|o||er |ec+uºe |,pop|çreu|ed ºpo|º c+u +||ºe +| º||eº W|e|e ||eºe
ºe|o|||e|c |e|+|oºeº |+.e |eeu |ero.ed.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 218
FICük£ 9-41 Seborrheìc keratoses, mu|tìp|e \u|||p|e ||oWu, W+||, p+pu|eº +ud uodu|eº ou ||e |+c|,
|+.|uç + 'ç|e+º,¨ |ee| +ud 'º|uc| ou¨ +ppe+|+uce. I||º p|c|u|e +|ºo º|oWº ||e e.o|u||ou o| ||e |eº|ouº. ||or ºr+||
ou|, º||ç|||, |+u, .e|, |||u p+pu|eº o| p|+queº |o |+|çe|, d+||e| uodu|+| |eº|ouº W||| + .e||ucouº ºu||+ce. ||+c||c+||,
+|| |eº|ouº ou ||e |+c| o| |||º e|de||, p+||eu| +|e ºe|o|||e|c |e|+|oºeº, W|+| ||e, |+.e |u corrou |º ||+| ||e, ç|.e
||e |rp|eºº|ou ||+| ||e, cou|d |e ºc|+ped o|| e+º||,, W||c|, |u |+c|, ||e, c+u.
FICük£ 9-40 Seborrheìc keratosìs â. 'r+||, |e+.||, p|çreu|ed ºe|o|||e|c |e|+|oºeº c+u |+.e + ºroo||
ºu||+ce +ud p|eºeu| + d|||e|eu||+| d|+çuoº||c c|+||euçe. p|çreu|ed |+º+| ce|| c+|c|uor+ +ud uodu|+| re|+uor+
|+.e |o |e e\c|uded. 8. |+|çe ºe|o|||e|c |e|+|oºeº |+.e + 'º|uc| ou¨ +ppe+|+uce, c+u |e .e|, d+|| +ud |||eçu|+|.
|ue |o ||e|| ru|||p||c||, ||e, uºu+||, do uo| p|eºeu| + d|+çuoº||c p|o||er. Aº º|oWu |e|e, ||e, c+u |e d|º||çu||uç.
â 8
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 219
FICük£ 9-42 8ecker oevus â. A º||ç|||, |+|ºed ||ç||·|+u p|+que W||| º|+|p|, de||ued +ud ||ç||, |||eçu|+|
|o|de| +ud |+|e|, .|º|||e |,pe||||c|oº|º ou ||e c|eº| o| + ¹o·,e+|·o|d r+|e p+||eu|. 8. |u |||º c+ºe o| Bec|e| ue.uº
||e r+ºº|.e |,pe||||c|oº|º couce+|º ||e |+u |+c|ç|ouud p|+que.
â 8
Bec|e| ue.uº (B|) |º + d|º||uc||.e +º,rp|o·
r+||c c||u|c+| |eº|ou ||+| |º + p|çreu|ed |+r+|·
|or+-|. e., + de.e|opreu|+| +uor+|, couº|º||uç
o| c|+uçeº |u p|çreu|+||ou, |+|| ç|oW||, +ud +
º||ç|||, e|e.+|ed ºroo|| .e||ucouº ºu||+ce (||ç.
9·+21 +ud 3 ).
|| occu|º roº||, |u r+|eº +ud |u +|| |+ceº. || +p·
pe+|º uo| +| ||||| |u| uºu+||, |e|o|e ¹5 ,e+|º o|
+çe +ud ºore||reº +||e| |||º +çe.
I|e |eº|ou |º p|edor|u+u||, + r+cu|e |u| W||| +
p+pu|+| .e||ucouº ºu||+ce uo| uu|||e ||e |eº|ou o|
+c+u||oº|º u|ç||c+uº. || |º ||ç|| ||oWu |u co|o| +ud
|+º + çeoç|+p||c p+||e|u W||| º|+|p|, der+|c+|ed
|o|de|º (||ç. 9·+2 1 ).
Corroueº| |oc+||ouº +|e ||e º|ou|de|º +ud ||e
|+c|. I|e |uc|e+ºed |+|| ç|oW|| |o||oWº ||e ouºe|
o| ||e p|çreu|+||ou +ud |º |oc+||/ed |o ||e +|e+º
||+| +|e p|çreu|ed. I|e p|çreu|+||ou |º |e|+|ed
|o |uc|e+ºed re|+u|u |u |+º+| ce||º +ud uo| |o +u
|uc|e+ºed uur|e| o| re|+uoc,|eº.
|| |º d|||e|eu||+|ed ||or + |+||, couçeu||+| re|+uo·
c,||c ue.uº, |ec+uºe B| |º uo| uºu+||, p|eºeu| +|
|||||, +ud ||or c+|e +u |+|| r+cu|eº |ec+uºe ||eºe
+|e uo| |+||,.
I|e |eº|ou e\|eudº |o| + ,e+| o| |Wo +ud ||eu
|er+|uº º|+||e, ou|, |+|e|, |+d|uç.
I|e|e |º .e|, |+|e|, |,pop|+º|+ o| uude||,|uç
º||uc|u|eº, e.ç., º|o||eu|uç o| ||e +|r o| |educed
||e+º| de.e|opreu| |u +|e+º uude| ||e |eº|ou.
\+u+çereu|. ||e |,pe||||c|oº|º c+u |e o| coº·
re||c couce|u |o ºore |ud|.|du+|º.
8£Ck£k N£vüS |C|·9 . 2¹o
°
|C|·¹0 . \3120/0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 220
I||c|oep|||e||or+º +|e |eu|çu +ppeud+çe |uro|º
W||| |+|| |u|| d|||e|eu||+||ou.
I|e |eº|ouº, W||c| +ppe+| +| pu|e||,, occu| ou
||e |+ce +ud |eºº o||eu ou ||e ºc+|p, uec|, +ud
uppe| ||uu| (||ç. 9·+! 1 ).
|eº|ouº r+, |e ou|, + |eW ºr+|| p|u| o| º||u·
co|o|ed p+pu|eº +| |||º| ç|+du+||, |uc|e+ºe |u
uur|e| +ud r+, |ecore qu||e |+|çe +ud |e
cou|uºed W||| BCC (||ç. 9·+! 1 ).
I||c|oep|||e||or+º c+u +|ºo +ppe+| +º ºo|||+|,
|uro|º, W||c| r+, |e uodu|+|, o| +ppe+| +º |||·
de||ued p|+queº |||e ºc|e|oº|uç BCC (||ç. 9·+! 3 ).

IkICh0£FIIh£II0MA |C|·9 . \3¹00/0 |C|·¹0 . |2!
',||uçor+º +|e |eu|çu +deuor+º o| ||e ec·
c||ue duc|º. I|e, +|e ¹· |o 2·rr, º||u·co|o|ed
o| ,e||oW, |||r p+pu|eº ||+| occu| roº||, |u
Woreu, |eç|uu|uç +| pu|e||,, ||e, r+, |e
|+r|||+|.
\oº| o||eu ru|||p|e |+||e| ||+u ºo|||+|,, ||e,
occu| roº| ||equeu||, ou |oWe| pe||o||||+| +|e+,
uºu+||, º,rre|||c+||, |u| +|ºo ou ||e e,e||dº
(||ç. 9·++) +ud ou ||e |+ce, +\|||+e, ur||||cuº,
uppe| c|eº|, +ud .u|.+.
I|e |eº|ouº |+.e + ºpec|||c ||º|o|oç|c p+||e|u.
r+u, ºr+|| duc|º |u ||e de|r|º W||| corr+·|||e
|+||º W||| ||e +ppe+|+uce o| '|+dpo|eº.¨
I|e |eº|ouº c+u |e d|º||çu||uç, +ud roº| p+||eu|º
W+u| ||er |ero.ed, |||º c+u |e doue e+º||, W|||
e|ec||oºu|çe|,.
S¥kINC0MA |C|·9 . 2¹o.0 ·2¹o.9
°
|C|·¹0 . |2!· \3+01/0
FICük£ 9-43 Irìchoepìthe|ìomas â. \u|||p|e, ºr+||, º|+|p|, de||ued ºroo|| p+pu|eº ||+| |oo| |||e e+||,
BCCº.
â
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 221
FICük£ 9-44 Syrìo¿omas ',rre|||c e|up||ou o| ¹· |o 2·rr º||u·co|o|ed, ºroo|| p+pu|eº ou ||e uppe|
+ud |oWe| e,e||dº.
FICük£ 9-43 Irìchoepìthe|ìomas (Cootìoued) 8. I||c|oep|||e||or+, ºo|||+|, |,pe. A uodu|+| |uro| ou ||e
uppe| ||p ||+| c+u |e cou|uºed W||| + |+º+| c+|c|uor+ o| ºqu+rouº ce|| c+|c|uor+.
8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 222
I|eºe +|e .e|, corrou |eº|ouº |u o|de| pe|ºouº
+ud +|e cou|uºed W||| ºr+|| BCCº. A|ºo occu|º
|u ºo||d o|ç+u ||+uºp|+u| |ec|p|eu|º ||e+|ed W|||
c,c|oºpo||ue. I|e |eº|ouº +|e ¹ |o ! rr |u d|+·
re|e| +ud |+.e |o|| |e|+uç|ec|+º|+ +ud ceu||+|
ur||||c+||ou (||ç. 9·+5).
IWo |e+|u|eº d|º||uçu|º| ºe|+ceouº |,pe|p|+º|+
||or uodu|+| BCC. (¹) ºe|+ceouº |,pe|p|+º|+ |º
ºo|| |o p+|p+||ou, uo| |||r +º |u uodu|+| BCC (uo|
ºupe|||c|+|), +ud (2) W||| |||r |+|e|+| corp|eºº|ou
|| |º o||eu poºº|||e |o e||c|| + .e|, ºr+|| ç|o|u|e o|
ºe|ur |u ||e .+||e, o| ||e ur||||c+|ed po|||ou o|
||e |eº|ou.
'e|+ceouº |,pe|p|+º|+º c+u |e deº||o,ed W|||
||ç|| e|ec||oc+u|e|,.
S£8AC£0üS h¥F£kFIASIA |C|·9 . 10o.9
I||º couçeu||+| r+||o|r+||ou o| ºe|+ceouº d||·
|e|eu||+||ou occu|º ou ||e ºc+|p o|, |+|e|,, ou ||e
|+ce (||ç. 9·+o).
A |+|||eºº, |||u, e|e.+|ed, ¹· |o 2·cr p|+que,
ºore||reº |+|çe|, W||| + c|+|+c|e||º||c o|+uçe
co|o| +ud + pe|||, o| W+||, ºu||+ce.
A|ou| ¹0° o| p+||eu|º c+u |e e\pec|ed |o de·
.e|op BCC |u ||e |eº|ou.
E\c|º|ou |º |ecorreuded +| +|ouud pu|e||, |o|
coºre||c |e+ºouº +ud |o p|e.eu| ||e occu||euce
o| BCC.
',¤¤¤,¤ . 0|ç+uo|d ue.uº.
N£vüS S£8AC£0üS |C|·9 . 2¹o.!
Aº ||e u+re ¤-.±º |rp||eº |||º |º + de.e|opreu·
|+| (|+r+||or+|ouº) d|ºo|de| c|+|+c|e||/ed |,
|,pe|p|+º|+ o| ep|de|r+| º||uc|u|eº (ep|de|r|º
+ud +due\+). I|e|e +|e uo ue.ore|+uoc,||c
ue.uº ce||º.
Ep|de|r+| ue.uº |º uºu+||, p|eºeu| +| ||||| o|
occu|º |u |u|+uc,, |+|e|,, || de.e|opº |u pu|e||,.
A|| ep|de|r+| ue.| ou ||e |e+d/uec| |eç|ou +|e
p|eºeu| +| |||||.
I|e|e +|e ºe.e|+| .+||+u|º o| ep|de|r+| ue.|. I|e
.-··±:¤±º -¤·!-·¤c| ¤-.±º r+, |e |oc+||/ed o|
ru|||p|e. I|e |eº|ouº +|e º||u·co|o|ed, ||oWu, o|
ç|+,|º|·||oWu (||ç. 9·+1) +ud +|e corpoºed o|
c|oºe|, ºe| .e||ucouº p+pu|eº, We|| c||curºc|||ed,
||e, +|e o||eu |u + ||ue+| +||+uçereu|-eºpec|+||,
ou ||e |eç-o| ||e, r+, +ppe+| |u B|+ºc||o ||ueº
ou ||e ||uu|. E\c|º|ou |º ||e |eº| ||e+|reu|, || |e+º·
|||e. B|opº, o| ||e |eº|ouº º|ou|d |e couº|de|ed |o
|u|e ou| BCC.
w|eu ||e |eº|ouº +|e e\|euº|.e ||e, +|e |e|red
º,º|-¤c|·c-! -¤·!-·¤c| ¤-.±º , +ud W|eu ||e,
+|e |oc+|ed ou |+|| ||e |od, ||e, +|e |e|red
¤-.±º ±¤·±º |c|-··º .
I|e |eº|ouº c+u e\||||| e|,||er+, ºc+||uç, +ud
c|uº||uç +ud +|e ||eu c+||ed ·¤||c¤¤c|¤·, |·¤-c·
.-··±:¤±º -¤·!-·¤c| ¤-.±º (||\E|). I|e |eº|ouº
ç|+du+||, eu|+|çe +ud |ecore º|+||e |u +do|eº·
ceuce.
I|e|e |º +|ºo + ¤¤¤·¤||c¤¤c|¤·, |·¤-c· .-··±:¤±º
-¤·!-·¤c| ¤-.±º (|||\E|).
E\|euº|.e ep|de|r+| ue.| ( -¤·!-·¤c| ¤-.±º º,¤·
!·¤¤- ) r+, |e ru|||º,º|er d|ºo|de|º +ud r+,
|e +ººoc|+|ed W||| de.e|opreu|+| +|uo|r+||||eº
(|oue c,º|º, |,pe|p|+º|+ o| |oue, ºco||oº|º, ºp|u+
||||d+, |,p|oº|º), .||+r|u |-|eº|º|+u| ||c|e|º, +ud
ueu|o|oç|c p|o||erº (reu|+| |e|+|d+||ou, ºe|·
/u|eº, co|||c+| +||op|,, |,d|ocep|+|uº). I|eºe
p+||eu|º |equ||e + corp|e|e e\+r|u+||ou, |uc|ud·
|uç ||e e,eº (c+|+|+c|º, op||c ue|.e |,pop|+º|+),
+ud c+|d|+c º|ud|eº |o |u|e ou| +ueu|,ºrº, p+|eu|
duc|uº +||e||oºuº.
£FI0£kMAI N£vüS |C|·9 . 2¹o
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 223
FICük£ 9-45 Sebaceous hyperp|asìa ¹· |o +·rr ºroo|| p+pu|eº W||| ceu||+| ur||||c+||ou ou ||e |o|e|e+d.
FICük£ 9-46 Nevus sebaceous Au e|e.+|ed
p|+que o| o|+uçe co|o| +ud pe|||, ºu||+ce. |o|e ||+|
||e |eº|ou |º |+|||eºº ou ||e ºc+|p.
FICük£ 9-4T £pìderma| oevus A ç|+,|º|
|||eçu|+| p|+que W||| + .e||ucouº ºu||+ce ou ||e e+|
e\|eud|uç ||ue+||, doWu |o ||e uec|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 224
£FI0£MI0I0C¥ AN0 £II0I0C¥
Veiy common, occuis mostly in adults. Females
> males.
£tìo|o¿y Unknown. It is consideied by many
to iepiesent a late histiocytic ieaction to an
aithiopod bite.
Skìo Iesìoos Usually asymptomatic papule oi
nodule (Fig. 9-49 À ), 3 to 10 mm in diametei.
Suiface vaiiably domed but may be depiessed
below plane of suiiounding skin. Textuie of
suiface may be dull, shiny, oi scaling. Top may
be ciusted oi scaiied secondaiy to excoiiation
oi shaving. Boideis ill defined, fading to noimal
skin. Co|or : vaiiable-skin-coloied, pink, biown,
tan, daik chocolate biown (Fig. 9-49 B ). Usually
daikei at centei, fading to noimal skin coloi at
maigin. Fiim. Dím¡|e sígn : lateial compiession
with thumb and index fingei pioduces a
depiession oi °dimple" (Fig. 9-49 C ).
DIstrIhutIvn Legs > aims > tiunk. Haidly
evei occuis on head, palms, soles. Usually soli-
taiy; may be multiple, iandomly scatteied.
8£NICN 0£kMAI AN0 Sü8CüIAN£0üS N£0FIASMS
AN0 h¥F£kFIASIAS
||por+º +|e º|uç|e o| ru|||p|e, |eu|çu ºu|cu|+ue·
ouº |uro|º ||+| +|e e+º||, |ecoçu|/ed |ec+uºe
||e, +|e ºo||, |ouuded, o| |o|u|+|ed +ud ro.+||e
+ç+|uº| ||e o.e||,|uç º||u (||ç. 9·+3 1 3 ).
\+u, ||por+º +|e ºr+|| |u| r+, +|ºo eu|+|çe |o >
o cr.
I|e, occu| eºpec|+||, ou ||e uec|, ||uu|, +ud ou
||e e\||er|||eº (||ç. 9·+3) |u| c+u occu| +u,·
W|e|e ou ||e |od,.
||por+º +|e corpoºed o| |+| ce||º ||+| |+.e ||e
º+re ro|p|o|oç, +º uo|r+| |+| ce||º W||||u +
couuec||.e ||ººue ||+reWo||. Auç|o||por+º |+.e
+ .+ºcu|+| corpoueu| +ud r+, |e |eude| |u co|d
+r||eu| |erpe|+|u|e +ud W||| corp|eºº|ou.
Auç|o||por+º o||eu |equ||e e\c|º|ou, W|e|e+º
o||e| ||por+º º|ou|d |e e\c|ºed ou|, W|eu
couº|de|ed d|º||çu||uç. ||poºuc||ou c+u +|ºo |e
pe||o|red W|eu ||poºor+º +|e ºo|| +ud ||uº
|+.e ou|, + r|uo| couuec||.e ||ººue corpoueu|.
|c¤·|·c| |·¤¤¤c º,¤!·¤¤- , +u +u|oºor+| dor|·
u+u| ||+|| +ppe+||uç |u e+||, +du|||ood, couº|º|º o|
|uud|edº o| º|oW|, ç|oW|uç uou|eude| |eº|ouº.
1!·¤¤º·|cº !¤|¤·¤ºc , o| |-·:±¤ !·º-cº- , occu|º
|u Woreu |u r|dd|e +çe, ||e|e +|e ru|||p|e
|eude|, uo| c||curºc|||ed |u| |+||e| d|||uºe |+||,
depoº||º.
3-¤·¸¤ º,¤¤-|··: |·¤¤¤c|¤º·º , W||c| +||ec|º r|d·
d|e·+çed reu, couº|º|º o| r+u, |+|çe uou|eude|,
co+|eºceu| poo||, c||curºc|||ed ||por+º, roº||,
ou ||e ||uu| +ud uppe| e\||er|||eº, ||e, co+|eºce
ou ||e uec| +ud r+, |e+d |o + '|o|ºe·co||+|¨ +p·
pe+|+uce.
IIF0MA |C|·9 . 2¹+
°
|C|·¹0 . |¹1· \3350/0
A de|r+|o||||or+ |º + .e|, corrou, |u||ou·|||e
de|r+| uodu|e, uºu+||, occu|||uç ou ||e e\||er|·
||eº.
|rpo||+u| ou|, |ec+uºe o| ||º coºre||c +ppe+|·
+uce o| ||º |e|uç r|º|+|eu |o| o||e| |eº|ouº,
ºuc| +º r+||çu+u| re|+uor+ W|eu || |º p|ç·
reu|ed.
k+|e|,, ||e |eº|ou r+, |e |eude|.
',¤¤¤,¤º . 'o|||+|, ||º||oc,|or+, ºc|e|oº|uç |e·
r+uç|or+.
0£kMAI0FI8k0MA |C|·9 . 2¹o
°
|C|·¹0 . |2!· \33!2/0
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 225
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Whoiling fascicles of spin-
dle cells with small amounts of pale blue cy-
toplasm and elongated nuclei. Some tumois
extend to the panniculus. Pigmented deimatofi-
biomas (Fig. 9-49 B ) contain lipids oi hemosi-
deiin pigment in the histiocytes in addition to
hypeipigmentation of the epideimis. Oveilying
epideimis fiequently hypeiplastic.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical findings-°dimple" sign (Fig. 9-49 C ),
but theie aie othei lesions that can iesult in
depiession with lateial piessuie, e.g., papu-
lonodulai lesions containing mucin, scai, blue
nevus, pilai cyst, metastatic caicinoma, Kaposi
saicoma, deimatofibiosaicoma piotubeians.
FICük£ 9-48 Iìpoma â. we||·de||ued, ºo||, |ouuded |uro|º |u ||e ºu|cu||º, ro.+||e |o|| +ç+|uº| ||e
o.e||,|uç º||u +ud ||e uude||,|uç º||uc|u|eº, |u + 5o·,e+|·o|d r+|e p+||eu|. |u |||º p+||eu| |eº|ouº We|e º,rre|||c
+ud We|e +|ºo |ouud ou ||e ||uu| +ud uppe| e\||er|||eº. 8. \u|||p|e ||por+º ou ||e |oWe| +|r o| + 50·,e+|·o|d
p+||eu|. I|eºe |eº|ouº We|e +|ºo º,rre|||c.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 226
C0ükS£ AN0 Fk0CN0SIS
Lesions appeai giadually ovei seveial months,
may peisist without inciease in size foi yeais to
decades, and may iegiess spontaneously.
MANAC£M£NI
Suigical iemoval is not usually indicated, as the
iesulting scai is often less cosmetically accept-
able. Ciyosuigeiy with a cotton-tip applicatoi
is often effective and pioduces a cosmetically
acceptable scai in most patients.
â
8 C
FICük£ 9-49 0ermatoIìbroma â. A dore·º|+ped, º||ç|||, e|,||er+|ouº +ud |+u uodu|e W||| + |u||ou·
|||e, |||r couº|º|euc,. 8. I||º |eº|ou |º p|çreu|ed. C+u |e cou|uºed W||| ||ue ue.uº o| e.eu uodu|+| re|+uor+.
I|e p|çreu| |º re|+u|u +ud |eroº|de||u. C. '||rp|e º|çu.¨ ||rp||uç o| ||e |eº|ou |º ºeeu W|eu p|uc|ed |e|Weeu
|Wo ||uçe|º.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 22T
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset Thiid decade, but all ages.
Sex Equal incidence in males and females.
kace Much moie common in blacks and in
peisons with blood gioup A.
£tìo|o¿y Unknown. They usually follow injuiy
to skin, i.e., suigical scai, laceiation, abiasion,
ciyosuigeiy, and electiocoagulation as well as
vaccination, acne, etc. Ke|oíJ may a|so aríse
s¡onìaneous|y, wíì|ouì |ísìory o[ ín,ury, usua||y
ín ¡resìerna| síìe.
CIINICAI MANIF£SIAII0N
Skìo Symptoms Usually asymptomatic. May
be piuiitic oi painful if touched.
Skìo Iesìoos Papules to nodules (Fig. 9-50 À , B )
to laige tubeious lesions. Most often the coloi
of the noimal skin but also biight ied oi bluish.
May be lineai aftei tiaumatic oi suigical injuiy
(Fig. 9-50 À ). Hypeitiophic scais tend to be
elevated and aie confined to appioximately
the site of the oiiginal injuiy (Fig. 9-50).
Keloids, howevei, may extend in a clawlike
fashion fai beyond any slight oiiginal injuiy
(Figs. 9-51, 9-52 À ) oi may be nodulai; tumoi-
like. Fiim to haid; may be tendei, suiface
smooth. Spontaneous keloids aiise de novo
without tiauma oi suigeiy, and usually occui
on the chest (Fig. 9-52 B ).
DIstrIhutIvn Eailobes, shouldeis, uppei back,
chest.
n,pe|||op||c ºc+|º +ud |e|o|dº +|e e\u|e|+u|
||||ouº |ep+|| ||ººueº +||e| + cu|+ueouº |uju|,.
A |,¤-·|·¤¤|·: º:c· |er+|uº cou||ued |o ||e º||e
o| o||ç|u+| |uju|,.
A |-|¤·! , |oWe.e|, e\|eudº |e,oud |||º º||e, o||eu
W||| c|+W|||e e\|euº|ouº.
\+, |e coºre||c+||, .e|, uuº|ç|||, +ud poºe +
ºe||ouº p|o||er |o| ||e p+||eu| || ||e |eº|ou |º
|+|çe +ud ou ||e e+| o| |+ce o| o.e| + jo|u|.
h¥F£kIk0FhIC SCAkS AN0 k£I0I0S |C|·9 . 10¹.+
°
|C|·¹0 . |9¹.0
FICük£ 9-50 hypertrophìc scar â. A ||o+d, |+|ºed ºc+| de.e|op|uç +| ||e º||e o| ºu|ç|c+| |uc|º|ou W||| |e|·
+uç|ec|+||c ||ood .eººe|º +ud + º||u, +||op||c ep|de|r|º. 8. \u|||p|e |,pe|||op||c ºc+|º ou ||e c|eº| o| +
22·,e+|·o|d r+|e W||| + ||º|o|, o| ºe.e|e +cue couç|o|+|+.
â
8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 228
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y HypertrvphIc Scur
Whoils of young fibious tissue and fibioblasts
in haphazaid aiiangement.
Ke|vId Featuies of hypeitiophic scai with
added featuie of thick, eosinophilic, acellulai
bands of collagen.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical diagnosis; biopsy not waiianted un-
less theie is clinical doubt, because this may
induce new hypeitiophic scaiiing. Diffei-
ential diagnosis includes deimatofibioma,
deimatofibiosaicoma piotubeians, desmoid
tumoi, scai with saicoidosis, foieign-body
gianuloma.
C0ükS£ AN0 Fk0CN0SIS
Hypeitiophic scais tend to iegiess, in time be-
coming flattei and softei. Keloids, howevei, may
continue to expand in size foi decades.
MANAC£M£NI
This is a ieal challenge, as no tieatment is highly
effective.
Iotra|esìooa| C|ucocortìcoìds Intialesional in-
jection of tiiamcinolone (10-40 mg/mL) eveiy
month may ieduce piuiitus oi sensitivity of
lesion, as well as ieduce its volume and flatten
it. This woiks quite well in small hypeitiophic
scais but less well in keloids. Can be combined
with ciyotheiapy wheieby the lesion is initially
fiozen with liquid nitiogen, allowed to thaw,
and then injected with tiiamcinolone (10-40
mg/mL). Aftei fieezing, the lesion becomes
edematous and is much easiei to inject.
Sur¿ìca| £xcìsìoo Lesions that aie excised sui-
gically often iecui laigei than the oiiginal
lesion. Excision with immediate postsuigical
iadiotheiapy is beneficial.
Sì|ìcooe Cream aod Sì|ìcooe Ce| Sheet
Repoited to be beneficial in keloids and is
painless and noninvasive. Not veiy effective in
authois` expeiience.
Freveotìoo Individuals pione to hypeitiophic
scais oi keloids should be advised to avoid
cosmetic pioceduies such as eai pieicing. Scais
fiom buins tend to become hypeitiophic. Can
be pievented by compiession gaiments.
FICük£ 9-51 ke|oìds we||·de||ued |||eçu|+| uodu|eº, .e|, |+|d ou p+|p+||ou, |u ||e +u||cu|+| |eç|ou +ud
c|ee| o| + !0·,e+|·o|d r+u. I|e |eº|ouº ou ||e e+||o|e +|oºe +||e| p|e|c|uç, ||e |eº|ou ou ||e r+ud||u|+| |eç|ou
+||e| |uc|º|ou o| +u |u||+red c,º|.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 229
â
FICük£ 9-52 ke|oìds â. Ke|o|d +||e| + deep |u|u. |o|e º+uº+çe· +ud c|+W|||e e\|euº|ouº o| ||e |e|o|d |u|o
uo|r+| º||u. 8. 'pou|+ueouº |e|o|dº ||+| +|oºe W|||ou| +pp+|eu| c+uºe ou ||e c|eº| o| + ¹9·,e+|·o|d r+u.
8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 230
A |+|e |o|r o| ºupe|||c|+| ju.eu||e ||||or+|oº|º.
||eºeu||uç +º +º,rp|or+||c ||eº|·co|o|ed o| p|u|
|||r uodu|e ou ||uçe|º +ud |oeº (||ç. 9·5!).
Appe+|º |u ||e |||º| ,e+| o| |||e, |eºº corrou|, |u
c|||d|ood.
n|º|o|oç|c+||, |u|e||+c|uç |uud|eº o| r,o||||o|·
|+º|º W||| eoº|uop||||c |uc|uº|ouº.
Beu|çu. 'pou|+ueouº |eç|eºº|ou |º |+|e. I|e+|reu|
|º ºu|ç|c+|
',¤¤¤,¤. k,e |uro|.
INFANIII£ 0ICIIAI FI8k0MAI0SIS |C|·9 . 151.!
°
|C|·¹0 . \12
FICük£ 9-53 IoIaotì|e dì¿ìta| Iìbromatosìs
A We||·de||ued p|u| uodu|e ou ||e ||uçe| o| +u
|u|+u|. uºu+||, ||e ||||d |o ||||| d|ç||º +|e +||ec|ed.
ne|e, ||e |uro| |º |ouud ou ||e ºecoud d|ç||.
S£CII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 231
FICük£ 9-54 Skìo ta¿s 'o|| º||u·co|o|ed +ud |+u peduucu|+|ed p+p|||or+º. I|eºe +|e .e|, corrou |u ||e
e|de||, o|eºe +ud +|e o|||ç+|o|, |eº|ouº |u +c+u||oº|º u|ç||c+uº, +º |u |||º p+||eu|.
A º||u |+ç |º + .e|, corrou, ºo||, º||u·co|o|ed o|
|+u o| ||oWu, |ouud o| o.+|, peduucu|+|ed p+p||·
|or+ (po|,p) (||ç. 9·5+), || |º uºu+||, couº|||c|ed
+| ||e |+ºe +ud r+, .+|, |u º|/e ||or >¹ rr |o
+º |+|çe +º ¹0 rr.
n|º|o|oç|c ||ud|uçº |uc|ude + |||uued ep|de|r|º
+ud + |ooºe ||||ouº ||ººue º||or+.
uºu+||, +º,rp|or+||c |u| occ+º|ou+||, r+, |e·
core |eude| |o||oW|uç ||+ur+ o| |o|º|ou +ud r+,
|ecore c|uº|ed o| |ero|||+ç|c.
|| occu|º ro|e o||eu |u ||e r|dd|e +çed +ud |u ||e
e|de||,.
\o|e corrou |u |er+|eº +ud |u o|eºe p+||eu|º
+ud roº| o||eu uo|ed |u |u|e||||ç|uouº +|e+º (+\||·
|+e, |u||+r+rr+|,, ç|o|u) +ud ou ||e uec| +ud
e,e||dº.
|| occu|º |u +c+u||oº|º u|ç||c+uº +ud re|+|o||c
º,ud|ore.
\+, |e cou|uºed W||| + peduucu|+|ed ºe|o|·
||e|c |e|+|oº|º, de|r+| o| corpouud re|+uoc,||c
ue.uº, ºo|||+|, ueu|o||||or+, o| ro||uºcur cou·
|+ç|oºur.
|eº|ouº |eud |o |ecore |+|çe| +ud ro|e uure|·
ouº o.e| ||re, eºpec|+||, du||uç p|eçu+uc,. |o|·
|oW|uç ºpou|+ueouº |o|º|ou, +u|o+rpu|+||ou c+u
occu|.
\+u+çereu| |º +ccorp||º|ed W||| º|rp|e ºu|p·
p|uç W||| ºc|ººo|º, e|ec||odeº|cc+||ou, o| c|,oºu|·
çe|,.
',¤¤¤,¤º Ac|oc|o|dou, cu|+ueouº p+p|||or+,
ºo|| ||||or+.
SkIN IAC |C|·9 . 10¹.9
°
|C|·¹0 . |9¹.3
232
S E C I | 0 N 1 0
I|e |e|r ¤|¤|¤º-¤º·|·.·|, deºc|||eº +u +|·
uo|r+| |eºpouºe |o ||ç||, uºu+||, ºuu||ç||,
occu|||uç W||||u r|uu|eº, |ou|º, o| d+,º o|
e\poºu|e +ud |+º||uç up |o Wee|º, rou||º,
+ud e.eu |ouçe|. Cu|+ueouº p|o|oºeuº|||.||,
|e+c||ouº |equ||e +|ºo|p||ou o| p|o|ou eue|ç,
|, +pp|op||+|e|, º|+ped ro|ecu|eº |e+d|uç |o
ro|ecu|+| de|o|r||,. Eue|ç, |º e|||e| d|ºpe|ºed
|+|r|eºº|, o| |º d||ec|ed |o c|er|c+| |e+c||ouº
||+| |e+d |o ro|ecu|+|, ce||u|+|, +ud ||ººue
d+r+çe |eºu|||uç |u c||u|c+| d|ºe+ºe. A|ºo|||uç
ro|ecu|eº c+u |e (¹) e\oçeuouº +çeu|º +p·
p||ed |op|c+||, o| º,º|er|c+||,, (2) eudoçeuouº
ro|ecu|eº e|||e| uºu+||, p|eºeu| |u º||u o|
p|oduced |, +u +|uo|r+| re|+|o||ºr, o| (!)
+ cor||u+||ou o| e\oçeuouº +ud eudoçeuouº
ro|ecu|eº ||+| |+.e +cqu||ed +u||çeu|c p|op·
e|||eº +ud ||uº e||c|| + p|o|o|+d|+||ou·d||.eu
|rruue |e+c||ou. ||¤|¤º-¤º·|·.·|, !·º¤·!-·º
¤::±· ¤¤|, ·¤ |¤!, ·-¸·¤¤º -·¤¤º-! |¤ º¤|c·
·c!·c|·¤¤ (|r+çe ¹0·¹).
I|e|e +|e |||ee ||o+d |,peº o| c:±|- ¤|¤·
|¤º-¤º·|·.·|,.
¹. A º±¤|±·¤·|,pe |eºpouºe W||| ||e de.e|opreu|
o| ro|p|o|oç|c º||u c|+uçeº º|ru|+||uç + uo|r+|
ºuu|u|u W||| e|,||er+, eder+, +ud |u||+e, ºuc|
+º |u p|o|o|o\|c |e+c||ouº |o d|uçº o| p|,|op|o·
|ode|r+||||º.
2. A ·cº| |eºpouºe |o ||ç|| e\poºu|e W||| de.e|op·
reu| o| .+||ed ro|p|o|oç|c e\p|eºº|ouº. r+cu|eº,
p+pu|eº, o| p|+queº, +º |u ec/er+|ouº de|r+||||º.
I|eºe +|e uºu+||, p|o|o+||e|ç|c |u u+|u|e o| |e·
|ouç |o ||e ºo·c+||ed |d|op+|||c p|o|ode|r+|oºeº
ºuc| +º po|,ro|p|ouº ||ç|| e|up||ou.
!. |·|·:c··c| |eºpouºeº +|e |,p|c+| |o| ºo|+| u|||c+||+,
u|||c+||+| |eº|ouº c+u +|ºo occu| |u e|,|||opo|e||c
po|p|,||+.
C|·¤¤·: ¤|¤|¤º-¤º·|·.·|,. c||ou|c |epe+|ed
ºuu e\poºu|eº o.e| ||re |eºu|| |u po|,ro|p||c
º||u c|+uçeº ||+| |+.e |eeu |e|red !-·¤c|¤·
|-|·¤º·º, o| p|o|o+ç|uç. A c|+ºº|||c+||ou o| º||u
|e+c||ouº |o ºuu||ç|| |º º|oWu |u I+||e ¹0·¹.
SkIN k£ACII0NS I0 SüNIIChI |C|·9 . o92.10
°
|C|·¹0 . |5o.3
8ASICS 0F CIINICAI Fh0I0M£0ICIN£
The main culpiit of solai iadiation-induced
skin pathology is the ultiaviolet poition of the
solai spectium. Ultiaviolet iadiation (UVR) in
photomedicine is divided into two piincipal
types: UVB (290-320 nm), the °sunbuin spec-
tium," and UVA (320-400 nm). UVA is subdi-
vided into UVA-1 (340-400 nm) and UVA-2
(320-340 nm). The unit of measuiement of
sunbuin is the mínímum eryì|ema Jose (MED),
which is the minimum ultiaviolet exposuie
that pioduces a cleaily maiginated eiythema in
the iiiadiated site 24 h aftei a single exposuie.
The MED is expiessed as the amount of eneigy
deliveied pei unit aiea: mJ/cm
2
(UVB) oi J/cm
2
(UVA). The MED foi UVB in Caucasians is
Fh0I0S£NSIIIvII¥,
Fh0I0-IN0üC£0 0IS0k0£kS,
AN0 0IS0k0£kS 8¥ I0NIIINC
kA0IAII0N
20-40 mJ/cm
2
(foi a skin phototype I oi II,
about 20 min in noithein latitudes at noon in
June) and foi UVA is 15-20 J/cm
2
(about 120
min in noithein latitudes at noon in June).
UVB eiythema develops in 6-24 h and fades
within 72-120 h. UVA eiythema develops in
4-16 h and fades within 48-120 h.
varìatìoos ìo Suo keactìvìty ìo Norma| Fersoos:
Fìttpatrìck Skìo Fhototypes (Table 10-2)
Sunbuin is seen most fiequently in individuals
who have pale white oi white skin and a limited
capacity to develop [atu|ìaìí·e, oi inducible,
melanin pigmentation (tanning) aftei exposuie
to UVR. Basic skin coloi ( tonsìíìuìí·e melanin
pigmentation) is divided into white, biown,
and black. Not all peisons with white skin have
the same capacity to develop tanning, and this
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 233
IMAC£ 10-1 \+||+||ouº |u ºo|+| e\poºu|e ou d|||e|eu| |od, +|e+º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 234
fact is the piincipal basis foi the classification of
°white" peisons into foui s|ín ¡|oìoìy¡es (SPT).
The SPT is based on the basic skin coloi (Table
10-2) and on a ¡erson's own esìímaìe of sun-
buining and tanning. One question peimits the
identification of the SPT: °Do you tan easily:"
Peisons with SPT I oi II will say immediately,
°No," and those with SPT III oi IV will say,
°Yes." Peisons with SPT I oi II aie iegaided as
°melanocompiomised," and those with SPT III
oi IV as °melanocompetent."
SPT I peisons usually have pale white skin
coloi, blond oi ied haii, and blue eyes; but, in
fact, they may have daik biown haii and biown
eyes, while theii skin coloi is pale white. SPT
I peisons sunbuin easily with shoit exposuies
and do not tan.
SPT II peisons aie a subgioup of SPT I and
sunbuin easily but ìan wíì| Jí[[ítu|ìy, wheieas
SPT III peisons may have some sunbuin with
shoit exposuies but can develop maiked tan-
ning. It is estimated that about 25% of white-
skinned peisons in the United States aie SPT I
and II. SPT IV peisons tan with ease and do not
sunbuin with shoit exposuies. SPT IV peisons
may have blond haii and blue eyes but moie
often have biown haii and biown eyes and light
tan (beige) constitutive skin coloi. Peisons with
constitutive biown skin aie teimed SPT V and
with black skin SPT VI. Note that sunbuin de-
pends on the amount of UVR eneigy absoibed.
Thus, with excessive sun exposuie, even SPT VI
peison can have a sunbuin.
||o|o|o\|c||,
'uu|u|u
||uç·/c|er|c+|·|uduced
||+u|·|uduced (p|,|op|o|ode|r+||||º)
||o|o+||e|ç,
||uç·/c|er|c+|·|uduced
C||ou|c +c||u|c de|r+||||º
'o|+| u|||c+||+
|d|op+|||c
|o|,ro|p|ouº ||ç|| e|up||ou
Ac||u|c p|u||ço
c
n,d|o+ .+cc|u||o|re
c
\e|+|o||c +ud uu|||||ou+|
|o|p|,||+ cu|+ue+ |+|d+
\+||eç+|e po|p|,||+
E|,|||opo|e||c p|o|opo|p|,||+
|e||+ç|+
c
||A·de||c|eu| p|o|ode|r+|oºeº
/e|ode|r+ p|çreu|oºur
c
0||e| |+|e º,ud|oreº
c
||o|oe\+ce||+|ed de|r+|oºeº
C||ou|c p|o|od+r+çe
|e|r+|o|e||oº|º (p|o|o+ç|uç)
'o|+| |eu||ço
Ac||u|c |e|+|oºeº
'||u c+uce|
|
c
Coud|||ouº uo| de+|| W||| |e|e +ud ||e |e+de| |º |e|e||ed |o K wo|||
e| +| (edº). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- 1|| ed.
|eW \o||, \cC|+W·n|||, 2003.
|
|o| co.e|+çe o| º||u c+uce|, ºee 'ec||ouº ¹¹ +ud ¹2.
IA8I£ 10-1 Simp|ified C|assification of
Skin Reactions to Sun|i¿ht
IA8I£ 10-2 C|assification of |ittpatrick's Skin Phototypes (SPI)
SPT 8as|c Sk|o 0o|or 8espoose to S0o £xpos0re
| |+|e W|||e |o uo| |+u, |u|u e+º||,
|| w|||e I+u W||| d||||cu||,, |u|u e+º||,
||| w|||e I+u e+º||, |u| r+, |u|u |u|||+||,
|\ ||ç|| ||oWu/o||.e I+u e+º||,, |+|d|, |u|u
\ B|oWu I+u e+º||,, uºu+||, do uo| |u|u
\| B|+c| Becore d+||e|, do uo| |u|u
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 235
'uu|u|u |º +u +cu|e, de|+,ed, +ud ||+uº|eu| |u||+r·
r+|o|, |eºpouºe o| uo|r+| º||u +||e| e\poºu|e |o
u\k ||or ºuu||ç|| o| +|||||c|+| ºou|ceº.
B, u+|u|e || |º + p|o|o|o\|c |e+c||ou.
'uu|u|u |º c|+|+c|e||/ed |, e|,||er+ (||ç. ¹0·¹ 1 )
+ud, || ºe.e|e, |, .eº|c|eº +ud |u||+e, eder+,
|eude|ueºº, +ud p+|u (||ç. ¹0·¹ 3 ).
ACüI£ SüN 0AMAC£ (SüN8ükN) |C|·9 . o92.1¹
°
|C|·¹0 . |55
£FI0£MI0I0C¥
Sunbuin depends on the amount of UVR
eneigy deliveied and the susceptibility of the
individual (SPT). It will theiefoie occui moie
often aiound midday, with decieasing latitude,
incieasing altitude, and decieasing SPT. Thus,
the °ideal" setting foi a sunbuin to occui would
be an SPT I individual (highest susceptibil-
ity) on Mt. Kenya (high altitude, close to the
equatoi) at noon (UVR is highest). Of couise,
sunbuin can occui at any latitude, but the
piobability foi it to occui decieases with in-
cieasing distance fiom the equatoi. Sunbuin is
seen moie often in those who fiequent beaches
oi tiavel to sunny vacation aieas. Sunbuin also
incieases with iespect to othei ambient con-
ditions, such as UVR ieflectance fiom snow,
watei, oi a glaciei.
A¿e Veiy young childien and eldeily peisons
aie said to have a ieduced capacity to sunbuin,
although this has not been thoioughly docu-
mented.
FAIh0C£N£SIS
The chiomophoies (molecules that absoib
UVR) foi UVB sunbuin eiythema aie not
known, but damage to DNA may be the in-
itiating event. The damage to DNA iesults
in excision of pyiimidine dimeis, and that
itself initiates a piotective tanning iesponse.
The mediatois that cause the eiythema in-
clude histamine foi both UVA and UVB.
In UVB eiythema, othei mediatois include
tumoi neciosis factoi (TNF- ), seiotonin,
piostaglandins, nitiic oxide, lysosomal en-
zymes, and kinins. The cytokine TNF- can
be detected as eaily as 1 h aftei exposuie.
The iesolution of eiythema is associated with
inteileukin (IL) 10, IL-4, and tiansfoiming
giowth factoi
1
.
CIINICAI MANIF£SIAII0N
Exposuie to the sun oi an aitificial UV souice.
Onset of symptoms depends on intensity of
exposuie; eiythema develops aftei 6 h and
peaks aftei 24 h.
Skìo Symptoms Piuiitus may be seveie even in
mild sunbuin; pain and tendeiness occui with
seveie sunbuin.
Coostìtutìooa| Symptoms Headache, chills, fe-
veiishness, and weakness aie not infiequent
in seveie sunbuin; some SPT I and II peisons
develop headache and malaise even aftei shoit
exposuies.
Ceoera| Appearaoce In seveie sunbuin, the
patient is °toxic"-with fevei, weakness, lassi-
tude, and a iapid pulse iate.
Skìo Iesìoos Confluent biight eiythema
always confined to sun-exposed aieas and thus
shaiply maiginated at the boidei between
exposed and coveied skin (Fig. 10-1À). Edema,
vesicles, and even bullae; always unifoim
eiythema and no °iash," as occuis in most
photoalleigic ieactions. Edematous aieas aie
iaised and tendei. As edema and eiythema fade
vesicles and blisteis diy to ciusts, which aie
then shed (Fig. 10-1 B ).
DIstrIhutIvn Stiictly confined to aieas of
exposuie; sunbuin can occui in aieas coveied
with clothing, depending on the degiee of UV
tiansmission thiough clothing, the level of
exposuie, and the SPT of the peison.
Mucous Membraoes Sunbuin of the tongue
can occui iaiely in mountain climbeis who
hold theii mouth open °panting"; it is fiequent
on the veimilion boidei of the lips.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y °Sunbuin" cells in the
epideimis (apoptotic keiatinocytes); also,
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 236
exocytosis of lymphocytes, vacuolization of
melanocytes and Langeihans cells. Dermís : en-
dothelial cell swelling of supeificial blood ves-
sels. Moie piominent with UVA eiythema,
with a densei mononucleai infiltiate and moie
seveie vasculai changes.
Sero|o¿y aod hemato|o¿y To iule out systemic
lupus eiythematosus (SLE) obtain antinucleai
antibody (ANA) level. Leukopenia may be pie-
sent in SLE.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Histoiy of UVR exposuie and sites of ieaction
on exposed aieas. P|oìoìoxít eryì|ema : obtain
histoiy of medications that can induce photo-
toxic eiythema. SLE can cause a sunbuin-type
eiythema. Eryì|ro¡oíeìít ¡roìo¡or¡|yría causes
eiythema, vesicles, edema, puipuia, and, only
iaiely, uiticaiial wheals.
C0ükS£ AN0 Fk0CN0SIS
Sunbuin, unlike theimal buins, cannot be classi-
fied on the basis of depth, i.e., fiist-, second-,
and thiid-degiee. Thiid-degiee buins aftei UVR
do not occui, and none of the featuies of thiid-
degiee theimal buins aie seen: scaiiing, loss of
sensation, loss of sweating, haii loss. A peima-
nent ieaction fiom seveie ultiaviolet buins is
mottled depigmentation, piobably ielated to
the destiuction of melanocytes, and eiuptive
solai lentigines (see Fig. 10-22).
MANAC£M£NI
Freveotìoo Peisons with SPT I oi II should
avoid sunbathing, especially between 11 A.M.
and 2 P.M . Clothing: UV-scieening cloth gai-
ments. Theie aie now many highly effective
topical chemical filteis (sunscieens) in lotion,
gel, and cieam foimulations. It is still not cleai
whethei iegulai use of topical sunscieens can
pievent melanoma of the skin, but theie is
ieasonable pioof that topical sunscieens ieduce
the induction of solai keiatoses and, piobably,
squamous cell caicinoma.
Moderate Suoburo TvpIcu| Cool wet diess-
ings, topical glucocoiticoids.
SystemIc Acetylsalicylic acid, indomethacin,
NSAIDs.
Severe Suoburo Bed iest. If veiy seveie, a
°toxic" patient may iequiie hospitalization foi
fluid ieplacement, piophylaxis of infection, etc.
TvpIcu| Cool wet diessings, topical glucocoi-
ticoids.
SystemIc Oial glucocoiticoids aie often given,
but theii efficacy has not been established by
contiolled studies. Indomethacin.
I||º deºc|||eº ||e |u|e|+c||ou o| u\k W||| + c|er|c+|/
d|uç W||||u ||e º||u.
IWo rec|+u|ºrº +|e |ecoçu|/ed. ¤|¤|¤|¤··: ·-·
c:|·¤¤º W||c| +|e p|o|oc|er|c+| |e+c||ouº |e+d·
|uç |o º||u p+||o|oç,, +ud
¤|¤|¤c||-·¸·: ·-c:|·¤¤º W|e|e + p|o|o+||e|çeu |º
|o|red ||+| |u|||+|eº +u |rruuo|oç|c |eºpouºe
+ud r+u||eº|º |u º||u +º + |,pe |\ |rruuo|oç|c
|e+c||ou.
I|e r+|u c||u|c+| d|||e|euce |e|Weeu p|o|o|o\|c
+ud p|o|o+||e|ç|c e|up||ouº |º ||+| ||e |o|re|
r+u||eº|º |||e +u |||||+u| (|o\|c) cou|+c| de|r+||||º
o| ºuu|u|u +ud ||e |+||e| |||e +u +||e|ç|c ec/er+·
|ouº cou|+c| de|r+||||º (I+||e ¹0·!).
0küC-[Ch£MICAI-IN0üC£0 Fh0I0S£NSIIIvII¥ |C|·9 . o92.19
°
|C|·¹0 . |5o.0
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 23T
â
FICük£ 10-1 Acute suoburo â. |+|u|u|, |eude|, |||ç|| e|,||er+ W||| r||d eder+ o| ||e uppe| |+c| W|||
º|+|p der+|c+||ou |e|Weeu ||e ºuu·e\poºed +ud ºuu·p|o|ec|ed W|||e +|e+º. 8. +3 |ou|º +||e| +cu|e ºuu|u|u.
E|,||er+ |º |+d|uç +ud |||º|e|º |+.e d||ed |o c|uº|º.
8
IA8I£ 10-3 Characteristics of Phototo\icity and Photoa||er¿y
Phototox|c|ty Photoa||ergy
C||u|c+| p|eºeu|+||ou 'uu|u|u |e+c||ou. e|,||er+, Ec/er+|ouº |eº|ouº,
eder+, .eº|c|eº +ud |u||+e, p+pu|eº, .eº|c|eº,
||equeu||, |eºo|.eº W||| ºc+||uç, c|uº||uç,
|,pe|p|çreu|+||ou, |u|u|uç, uºu+||, p|u||||c
ºr+|||uç
n|º|o|oç, Apop|o||c |e|+||uoc,|eº, 'pouç|o||c de|r+||||º,
ºp+|ºe de|r+| |u|||||+|e o| deuºe, de|r+|
|,rp|oc,|eº, r+c|op|+çeº, |,rp|o||º||oc,||c
+ud ueu||op|||º |u|||||+|e
|+||op|,º|o|oç, |||ec| ||ººue |uju|, I,pe |\ de|+,ed
|,pe|ºeuº|||.||,
|epouºe
0ccu||euce +||e| \eº |o
|||º| e\poºu|e
0uºe| o| \|uu|eº |o |ou|º 2+-+3 |
e|up||ou +||e|
e\poºu|e
|oº+çe o| +çeu| |+|çe 'r+||
ueeded |o| e|up||ou
C|oºº·|e+c||.||, k+|e Corrou
W||| o||e| +çeu|º
||+çuoº|º C||u|c+| + p|o|o|eº|º C||u|c+| + p|o|o|eº|º
+ p|o|op+|c| |eº|º
Ad+p|ed ||or n ||r, |u K wo||| e| +| (edº). |·|c¤c|··:|'º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- 1|| ed. |eW \o||, \cC|+W·n|||, 2003.
I||º deºc|||eº +u +d.e|ºe |e+c||ou o| ||e º||u ||+|
|eºu||º ||or º|ru||+ueouº e\poºu|e |o ce||+|u
d|uçº (.|+ |uçeº||ou, |ujec||ou, o| |op|c+| +pp||c+·
||ou) +ud |o u\k o| .|º|||e ||ç||.
I|e c|er|c+|º r+, |e ||e|+peu||c, coºre||c,
|uduº|||+|, o| +ç||cu||u|+|.
I|e|e +|e |Wo |,peº o| |e+c||ou. (¹) º,º|er|c
p|o|o|o\|c de|r+||||º, occu|||uç |u |ud|.|du+|º
º,º|er|c+||, e\poºed |o + p|o|oºeuº|||/|uç +çeu|
(d|uç) +ud ºu|ºequeu| u\k, +ud (2) |oc+| p|o|o·
|o\|c de|r+||||º, occu|||uç |u |ud|.|du+|º |op|c+||,
e\poºed |o ||e p|o|oºeuº|||/|uç +çeu| +ud ºu|ºe·
queu| u\k.
Bo|| +|e -·c¸¸-·c|-! º±¤|±·¤ ·-º¤¤¤º-º (e|,·
||er+, eder+, .eº|c|eº, +ud/o| |u||+e).
',º|er|c p|o|o|o\|c de|r+||||º occu|º |u +|| |l|·
-·¤¤º-! º·|-º |oc+| p|o|o|o\|c de|r+||||º ou|, |u
||e |¤¤·:c| c¤¤|·:c|·¤¤ º·|-º
Fh0I0I0XIC 0küC-[Ch£MICAI-IN0üC£0 Fh0I0S£NSIIIvII¥ |C|·9 . o92.19
°
|C|·¹0 . |5o.0
238 FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E'
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 239
IA8I£ 10-4 Systemic Phototo\ic A¿ents
c
Property 6eoer|c hame Property 6eoer|c hame
Au||+u\|e|, d|uçº A|p|+/o|+r ||u|e||cº n,d|oc||o|o|||+/|de
C||o|d|+/epo\|de 0yatìde
Au||c+uce| d|uçº Ad||+r,c|u |,eº ||uo|eºce|u
|+c+||+/|ue \e||,|eue ||ue
||uo|ou|+c|| |u|ocour+||uº |ºo|+|euº
\e||o||e\+|e 5-Methoxypsora|eo
\|u||+º||ue 8-Methoxypsora|eo
Au||dep|eºº+u|º I||c,c||cº 4, 5', 8-Irìmethy|psora|eo
Ar||||p|,||ue n,poç|,cer|cº 'u||ou,|u|e+º.
|eº|p|+r|ue Ace|o|e\+r|de
|r|p|+r|ue C||o|p|op+r|de
Au|||uuç+|º C||ºeo|u|.|u C||p|/|de
Au||r+|+||+|º C||o|oqu|ue C|,|u||de
0u|u|ue Io|+/+r|de
Au||r|c|o||+|º 0u|uo|oueº Io|butamìde
C|p|o||o\+c|u |'A||º Ace||c +c|d de||.+||.e
Euo\+c|u ||c|o|eu+c
Cer|||o\+c|u Au|||+u|||c +c|d de||.+||.e
IomeI|oxacìo \e|eu+r|c +c|d
\o\|||o\+c|u Euo||c +c|d de||.+||.e.
Na|ìdìxìc acìd Fìroxìcam
|o|||o\+c|u ||op|ou|c +c|d de||.+||.eº
0||o\+c|u ||up|o|eu
SparI|oxacìo Ke|op|o|eu
'u||ou+r|deº Naproxeo
Ie||+c,c||ueº 0\+p|o/|u
0emec|ocyc|ìoe I|+p|o|eu|c +c|d
0oxycyc|ìoe '+||c,||c +c|d de||.+||.e
\|uoc,c||ue ||||uu|º+|
Ie||+c,c||ue 0||e|º
I||re||op||r Ce|eco\||
\o||cou+/o|e Nabumetooe
Au||pº,c|o||c ||euo|||+/|ueº ||o|od,u+r|c ForIìmer
d|uçº Ch|orpromatìoe ||e|+p, +çeu|º verteporIìo
|e|p|eu+/|ue ke||uo|dº Ac|||e||u
Froch|orperatìoe |ºo||e||uo|u
I||o||d+/|ue 0||e| ||u|+r|de
I||||uope|+/|ue n,pe||c|u
C+|d|+c Amìodarooe |,||do\|ue (.||+r|u B
o
)
red|c+||ouº 0u|u|d|ue k+u|||d|ue
||u|e||cº Furosemìde
I||+/|deº
Beud|o||ure|||+/|de
Ch|orothìatìde
c
Corrou|, |epo||ed d|uçº +|e p||u|ed |u |o|d.
'ou|ce. Ad+p|ed ||or n ||r, |u K wo||| e| +| (edº). |·|c¤c|··:|'º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- 1|| ed. |eW \o||, \cC|+W·n|||, 2003.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 240
£FI0£MI0I0C¥
Occuis in eveiyone aftei ingestion of a suf-
ficient dose of a photosensitizing diug and
subsequent UVR. Theiefoie all ages, both sexes,
all iaces, and all types of skin coloi. Phototoxic
diug ieactions aie moie fiequent than photoal-
leigic diug sensitivity.
£II0I0C¥ AN0 FAIh0C£N£SIS
Foimation of toxic photopioducts such as fiee
iadicals oi ieactive oxygen species such as sing-
let oxygen. The piincipal sites of damage aie
nucleai DNA oi cell membianes (plasma, lyso-
somal, mitochondiial). The action spectium
is UVA. Diugs eliciting systemic phototoxic
deimatitis aie listed in Table 10-4. Some diugs
causing phototoxic ieactions can also elicit pho-
toalleigic ieactions (see below).
CIINICAI MANIF£SIAII0N
An °exaggeiated sunbuin" aftei solai oi UVR
exposuie that norma||y wou|J noì e|ítíì a sun-
|urn ín ì|aì ¡arìítu|ar ínJí·íJua|. Occuis usu-
ally within houis aftei exposuie, with some
agents such as psoialens aftei 24 h, and peaking
at 48 h. Skin symptoms: buining, stinging,
piuiitus.
Skìo Iesìoos Ear|y. The skin lesions aie those
of an °exaggeiated sunbuin." Eiythema, edema
(Fig. 10-2 À ), and vesicle and bulla foimation
(Fig. 10-2 B ) confined exclusively to aieas
exposed to light. An eczematous ieaction is noì
seen in phototoxic ieactions.
Specìa| Freseotatìoos: Fseudoporphyrìa With
some diugs theie is little eiythema but pio-
nounced blisteiing and skin fiagility with
eiosions (see Fig. 22-13) and, upon iepeated
exposuies, healing milia foimation, paiticulaily
on the doisa of hands and lowei aims. Clini-
cally indistinguishable fiom poiphyiia cutanea
taida (see Fig. 10-11)-hence the teim ¡seu-
Jo¡or¡|yría (see Section 22).
Naì|s Subungual hemoiihage and photo-
onycholysis can occui with ceitain diugs
(psoialens, demethylchloitetiacycline, benoxa-
piofen).
Fì¿meotatìoo Maiked biown epideimal mela-
nin pigmentation may occui in the couise of
some eiuptions. With ceitain diugs especially
(chloipiomazine and amiodaione), a slate giay
deimal melanin pigmentation develops (see
Section 22).
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Inflammation, °sunbuin
cells" (apoptotic keiatinocytes) in the epi-
deimis, epideimal neciobiosis, intiaepideimal
and subepideimal vesiculation. Absence of ec-
zematous changes.
Fhototestìo¿ Foi veiification of the inciimi-
nating agent, template test sites aie exposed to
incieasing doses of UVA ( ¡|oìoìoxít reatìíons
are a|mosì a|ways Jue ìo UVÀ ) while patient is
on the diug. The UVA MED will be much lowei
than that foi noimal individuals of the same
skin phototype. Aftei diug is excieted and then
eliminated fiom the skin, a iepeat UVA pho-
totest will ieveal an íntrease in the UVA MED.
This test may be impoitant if patient is on
multiple potentially phototoxic diugs.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Histoiy of exposuie to diugs is most impoi-
tant, as aie the types of moiphologic changes
in the skin chaiacteiistic of phototoxic diug
eiuptions: confluent eiythema, edema, vesicles,
bullae. Diffeiential diagnosis includes iegulai
sunbuin, phototoxic ieactions due to excess of
endogenous poiphyiins, and photosensitivity
due to othei diseases, e.g., SLE.
C0ükS£ AN0 Fk0CN0SIS
Phototoxic diug sensitivity is a majoi pioblem,
since the abnoimal ieactions seiiously limit oi
exclude the use of impoitant diugs: diuietics, an-
tihypeitensive agents, diugs used in psychiatiy.
Wheieas, as a iule, phototoxicity occuis in piac-
tically anyone who is on a phototoxic diug-in
contiast to photoalleigy, which occuis only in
the sensitized-some individuals nonetheless
show phototoxic ieactions to a paiticulai diug
and otheis do not. It is not known why. Pho-
totoxic diug ieactions disappeai aftei cessation
of diug.
MANAC£M£NI
As foi sunbuin.
S¥SI£MIC Fh0I0I0XIC 0£kMAIIIIS |C|·9 . o92.19
°
|C|·¹0 . o5o.0
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 241
FICük£ 10-2 Fhototoxìc dru¿-ìoduced
photoseosìtìvìty â. \+ºº|.e eder+ +ud
e|,||er+ |u ||e |+ce o| + ¹1·,e+|·o|d ç|||
W|o W+º ||e+|ed W||| dere||,|c||o||e||+·
c,c||ue |o| +cue. |o|e +|ºeuce o| e|,||er+
||or uec|, W||c| W+º º|+ded 8. |uº|,
e|,||er+ W||| |||º|e||uç ou ||e do|º+ o| |o||
|+udº |u + p+||eu| ||e+|ed W||| p||o\|c+r.
ne|e ||e|e |º |u+d.e||eu| cou|+c| W||| o| ||e|+·
peu||c +pp||c+||ou o| + p|o|oºeuº|||/e|, |o||oWed |,
u\A |||+d|+||ou (p|+c||c+||, +|| |op|c+| p|o|oºeuº|·
||/e|º |+.e +u +c||ou ºpec||ur |u ||e u\A |+uçe).
I|e roº| corrou |op|c+| p|o|o|o\|c +çeu|º +|e
||º|ed |u I+||e ¹0·5, +ud ||e roº| corrou |ou|e
o| cou|+c| |º e|||e| ||e|+peu||c o| occup+||ou+|
e\poºu|e.
C||u|c+| p|eºeu|+||ou |º |||e +cu|e |||||+u| cou·
|+c| de|r+||||º (ºee 'ec||ou 2), W||| e|,||er+,
ºWe|||uç, .eº|cu|+||ou, +ud |||º|e||uç cou||ued |o
||e º||eº o| cou|+c| W||| ||e p|o|o|o\|c +çeu|.
',rp|orº +|e ºr+|||uç, º||uç|uç, +ud |u|u|uç
|+||e| ||+u ||c||uç.
ne+||uç uºu+||, |eºu||º |u p|ououuced p|çreu|+·
||ou. I|e roº| corrou +ud ||uº |rpo||+u| |op|·
c+| p|o|o|o\|c de|r+||||º |º p|,|op|o|ode|r+||||º,
deºc|||ed |e|oW.
I0FICAI Fh0I0I0XIC 0£MAIIIIS |C|·9 . o92.19
°
|C|·¹0 . |5o.0
â
8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 242
||,|op|o|ode|r+||||º (p|+u| + ||ç|| de|r+||||º)
|º +u |u||+rr+||ou o| ||e º||u c+uºed |, cou|+c|
W||| ce||+|u p|+u|º du||uç |ec|e+||ou+| o| occu·
p+||ou+| e\poºu|e |o ºuu||ç||.
I|e |u||+rr+|o|, |eºpouºe |º + p|o|o|o\|c |e·
+c||ou |o p|o|oºeuº|||/|uç c|er|c+|º |u ºe.e|+|
p|+u| |+r|||eº.
Corrou |,peº o| ||| +|e due |o e\poºu|e |o
||reº, ce|e|,, +ud re+doW ç|+ºº.
',¤¤¤,¤º Be||oque de|r+||||º, ||re de|r+||||º.
Fh¥I0Fh0I00£kMAIIIIS (FF0) |C|·9 . o92.12
°
|C|·¹0 . |5o.2
£FI0£MI0I0C¥ AN0 £II0I0C¥
Common. Usually in spiing and summei oi
all yeai in tiopical climates. PPD can occui at
any age.
kace All skin colois; biown- and black-
skinned peisons may develop only maiked
spotty daik pigmentation without eiythema oi
bullous lesions.
0ccupatìoo Celeiy pickeis, caiiot piocessois,
gaideneis ¦exposed to caiiot gieens oi to °gas
plant" ( Dítìamnus a||us )], and baitendeis (lime
juice) who aie exposed to sun in outside bais.
Nonoccupational: housewives and useis of pei-
fumes containing oil of beigamot; peisons in
holiday diinking time diinks oi eating oianges
in the sun.
£tìo|o¿y Phototoxic ieaction caused by pho-
toactive fuiocoumaiins (psoialens) contained
in the plants (Table 10-5).
CIINICAI MANIF£SIAII0N
The patient gives a histoiy of exposuie to ceitain
plants (lime, lemon, wild paisley, celeiy, giant
hogweed, paisnips, caiiot gieens, figs). Lime
juice is a fiequent cause: making lime diinks,
haii iinses with lime juice. Women who use
peifumes containing oil of beigamot (which
contains beigapten, 5-methoxypsoialen) may
develop stieaks of pigmentation only in aieas
wheie the peifume was applied, especially the
sides of the neck. This is called |er|oque Jerma-
ìíìís (Fiench: |er|oque, °pendant"). Peisons walk-
ing on beaches containing meadow giass and
childien playing in giassy meadows develop PPD
on the legs; meadow giass contains agiimony.
Skìo Symptoms Smaiting, sensation of sun-
buin, pain, latei piuiitus.
Skìo Iesìoos Acute: eiythema, edema, vesicles,
and bullae (Fig. 10-3). Lesions may appeai
pseudopapulai befoie vesicles aie evident. Often
bizaiie stieaks, aitificial patteins that indicate
an °outside job" (Fig. 10-4). Scatteied aieas on
the sites of contact, especially the aims, legs,
and face. Residual hypeipigmentation in bizaiie
stieaks (beiloque deimatitis) (Fig. 10-5).
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Easily made if the pattein is iecognized and a
caieful histoiy is taken. Diffeiential diagnosis is
piimaiily acute iiiitant contact deimatitis, with
stieaky pattein poison ivy deimatitis (see Figs.
2-8, 2-10), but this is eczematous.
C0ükS£
May be an impoitant occupational pioblem, as
in celeiy pickeis. The acute eiuption has a shoit
life and fades spontaneously, but the pigmenta-
tion may last foi many weeks.
MANAC£M£NI
Wet diessings may be indicated in the acute
vesiculai stage. Topical glucocoiticoids.
IA8I£ 10-5 Common Iopica| Phototo\ic A¿ents
Ageot £xpos0re
koºe Beuç+| 0p|||+|ro|oç|c e\+r|u+||ou
||uo|eºce|u |,e
|u|ocour+||uº 0ccu| u+|u|+||, |u p|+u|º (roº||, C¤¤¤¤º·|c- ºpp., |¤|·|·|-·c- ºpp., ||u||º +ud
.eçe|+||eº (||re, |erou, ce|e|,, ||ç, p+|º|e,, p+|ºu|p), uºed |u pe||ureº +ud
coºre||cº (e.ç., o|| o| |e|ç+ro|), +ud uºed |o| |op|c+| p|o|oc|ero||e|+p,
I+| Iop|c+| ||e|+peu||c +çeu|, |oo||uç r+|e||+|º, |o+d |+|||uç
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 243
FICük£ 10-4 Fhytophotodermatìtìs |u +
+3·,e+|·o|d r+u W|o W+º ºuu|+|||uç |u + re+doW.
|rred|+|e|, |e|o|e .eº|c|eº +ud |||º|e|º +||ºe e|,||er+·
|ouº |eº|ouº r+, +ppe+| |+|ºed, ç|.|uç ||e |+|ºe |rp|eº·
º|ou o| |e|uç p+pu|+|. |o|e º||e+|, p+||e|u.
FICük£ 10-5 8er|oque dermatìtìs I|e p+||eu|
|+d +pp||ed + ||+ç|+u| |+|| o|| |o |e| º|ou|de|º
+ud c|eº| |u| º|oWe|ed ou|, ||e ||ou| o| |e| |od,
|e|o|e ço|uç |u|o ||e ºuu. I|e |+|| o|| cou|+|ued o||
o| |e|ç+ro|, +ud p|çreu|+||ou |º uoW uo|ed W|e|e
|| |||c||ed doWu ||or ||e º|ou|de|º |o ||e |u||oc|º.
(Cou||eº, o| ||. I|or+º 'c|W+|/.)
FICük£ 10-3 Fhytophotodermatìtìs (p|aot
+ |ì¿ht): acute wìth b|ìsters I|eºe |u||+e We|e
||e |eºu|| o| e\poºu|e |o ur|||||e|+e +ud ||e ºuu.
I||º 50·,e+|·o|d |ouºeW||e W+º Weed|uç |e| ç+|deu
ou + ºuuu, d+,. ur|||||e|+e cou|+|u |e|ç+p|eu
(5·re||o\,pºo|+|eu), W||c| |º + po|eu| |op|c+|
p|o|o|o\|c c|er|c+|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 244
I||º |eºu||º ||or |u|e|+c||ou o| + p|o|o+||e|çeu
+ud u\A |+d|+||ou.
|u ºeuº|||/ed |ud|.|du+|º e\poºu|e |o + p|o|o+||e|·
çeu +ud ºuu||ç|| |eºu||º |u + p|u||||c ec/er+|ouº
e|up||ou cou||ued |o e\poºed º||eº +ud c||u|c+||,
|ud|º||uçu|º|+||e ||or +||e|ç|c cou|+c| de|r+||||º.
|u roº| p+||eu|º ||e e||c|||uç d|uç/c|er|c+| |+º
|eeu +pp||ed |op|c+||,, |u| º,º|er|c e||c||+||ou
+|ºo occu|º.
Fh0I0AII£kCIC 0küC-[Ch£MICAI-IN0üC£0 Fh0I0S£NSIIIvII¥ |C|·9 . o92.12
°
|C|·¹0 . |5o.¹
£FI0£MI0I0C¥
A¿e oI 0oset Moie common in adults.
kace All skin phototypes and colois.
Iocìdeoce Photoalleigic diug ieactions occui
much less fiequently than do phototoxic diug
ieactions.
£II0I0C¥ AN0 FAIh0C£N£SIS
Topically applied chemical/diug plus UVA
iadiation. The chemicals aie disinfectants, an-
timiciobials, agents in sunscieens, peifumes in
afteishaves, oi whiteneis (Table 10-6). The chemi-
cal agent piesent in the skin absoibs photons and
foims a photopioduct; this then binds to a soluble
oi membiane-bound piotein to foim an antigen
to which a type IV immune iesponse is elicited.
Since photoalleigy depends on individual im-
munologic ieactivity, it develops in only a small
peicentage of peisons exposed to diugs and
light and is elicited only in those who have been
sensitized. Photoalleigy can also be induced by
systemic administiation of a diug and elicited by
topical administiation of the same diug, and vice
veisa. UVA is always iequiied.
CIINICAI MANIF£SIAII0N
May be uncleai in that the initial exposuie
induces sensitization to delayed-type hypei-
sensitivity ieactions, and the eiuption occuis
only on subsequent exposuie. Topically applied
photosensitizeis aie the most fiequent cause of
photoalleigic eiuptions (Table 10-6). Eiuption
is highly piuiitic.
Skìo Iesìoos The moiphology of the skin
ieaction is much diffeient fiom that in phototoxic
diug sensitivity. Acute photoalleigic ieaction
patteins aie clinically indistinguishable fiom
alleigic contact deimatitis (Fig. 10-6): papulai,
vesiculai, scaling, and ciusted. Occasionally
theie can also be a lichenoid eiuption similai
to lichen planus. In chionic diug photoalleigy,
theie is scaling, lichenification, and maiked
IA8I£ 10-6 Iopica| Photoa||er¿ens

6ro0p 0hem|ca| hame
'uuºc|eeuº u\B +|ºo||e|º.
para-Amìoobeotoìc acìd
(FA8A)
C|uu+r+|eº
'+||c,|+|eº
u\A +|ºo||e|º.
Au|||+u||+|e
8eotopheoooes
||+ç|+uceº 6-Methy|coumarìo
Musk ambrette
'+ud+|Wood o||
Au|||+c|e||+|º 0ìbromosa|ìcy|aoì|ìde
Ietrach|orosa|ìcy|aoì|ìde
I||||oroº+||c,|+u|||de
C||o||e\|d|ue
||re||,|o|·d|re||,| |,d+u|o|u
ne\+c||o|op|eue
8ìthìooo|
||c||o|op|eue
I||c|oº+u
Su|Iooamìdes
Au|||uuç+|º I||o||ºc||o|op|euo|
Buc|oº+r|de
B|oroc||o|oº+||c,|+u|||de
0||e|º Ch|orpromatìoe
C||oqu|uo|
Ke|op|o|eu
0|+qu|udo\
||ore||+/|ue
0u|u|d|ue
I||ou|e+
c
Corrou|, |epo||ed d|uçº +|e p||u|ed |u |o|d.
'ou|ce. Ad+p|ed ||or n ||r, |u K wo||| e| +| (edº). |·|c¤c|··:|'º
|-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- 1|| ed. |eW \o||,
\cC|+W·n|||, 2003.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 245
piuiitus mimicking atopic deimatitis oi, again,
chionic alleigic contact deimatitis (Fig. 10-6;
see also °Eczema/Deimatitis," Section 2.)
DIstrIhutIvn Confined piimaiily to aieas ex-
posed to light (distiibution pattein of photo-
sensitivity), but theie may be spieading onto
adjacent nonexposed skin; theiefoie, it is not so
well ciicumsciibed as in phototoxic ieactions.
Of diagnostic help is the fact that in the face
the uppei eyelids, the aiea undei the nose, and
a thin stiip of skin between the lowei lip and
the chin aie often spaied (shaded aieas) (Fig.
10-6).
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Acute and chionic
delayed-type hypeisensitivity ieaction: epideimal
spongiosis with lymphocytic infiltiation.
0IACN0SIS
Histoiy of exposuie to diug is most impoitant,
as well as the types of moiphologic changes in
the skin: this is essentially an alleigic contact
deimatitis pattein. In essence, the diffeiential
diagnosis between this and phototoxic eiup-
tions is identical to that desciibed foi toxic/
iiiitant and alleigic contact deimatitis (see
Section 2).
Diagnosis iequiies the use of patch and
photopatch tests. Photopatch tests aie done in
duplicate because photoalleigens can also cause
contact hypeisensitivity. Photoalleigens aie ap-
plied to the skin and coveied. Aftei 24 h, one
set of the duplicate test sites is exposed to UVA
while the othei set iemains coveied; test sites
aie iead foi ieactions aftei 48-96 h. An eczema-
tous ieaction in the iiiadiated site but not in the
FICük£ 10-6 Fhotoa||er¿ìc dru¿-ìoduced photoseosìtìvìty I||º o0·,e+|·o|d r+|e º|oWº +u ec/er+|ouº
de|r+||||º |u ||e |+ce. ne W+º |+||uç |||re||op||r·ºu||+re||o\+/o|e. |o|e ºp+||uç o| e,e||dº (p|o|ec|ed |, ºuu·
ç|+ººeº), uude| ||e uoºe, +ud ||e +|e+ uude| ||e |oWe| ||p (º|+ded +|e+º)
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 246
noniiiadiated site confiims photoalleigy to the
paiticulai agent tested.
C0ükS£ AN0 Fk0CN0SIS
Photoalleigic deimatitis can peisist foi months
to yeais. This is known as ¡ersísìenì |íg|ì
reatìíon , oi t|ronít atìínít Jermaìíìís (Fig.
10-7À, B), The classic geneialized peisistent
light ieactions weie caused by exposuie to
soaps containing salicylanilides (Table 10-6).
In ¡ersísìenì |íg|ì reatìíon, the action spectium
usually bioadens to involve UVB, and the
condition peisists despite discontinuation of
the causative photoalleigen, with each new UV
exposuie aggiavating the condition. Chionic
eczema-like lichenified and extiemely itchy
confluent plaques iesult (Fig. 10-7À, B), which
lead to gioss disfiguiement and a distiessing
situation foi the patient. As the condition is
now independent of the oiiginal photoalleigen
and is aggiavated by each new solai exposuie,
avoidance of photoalleigen does not cuie the
disease. In contiast to eailiei belief, chionic
actinic deimatitis does not piogiess to lym-
phoma.
MANAC£M£NI
In seveie cases, immunosuppiession (azathio-
piine plus glucocoiticoids oi oial cyclospoiine)
is iequiied.
FICük£ 10-T 0ru¿-ìoduced photoseosìtìvìty: persìsteot |ì¿ht eruptìoo â. E|,||er+|ouº p|+queº cou·
||ued |o ||e |+ce +ud uec|, ºp+||uç ||e º|ou|de|º. I||º r+|e |+º e\c|uc|+||uç p|u|||uº.
â
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 24T
FICük£ 10-T 0ru¿-ìoduced photoseosìtìvìty: persìsteot |ì¿ht eruptìoo (Cootìoued ) 8. |e|º|º|eu| ||ç||
e|up||ou W||| |u|||||+|ed ec/er+|ouº e|up||ou |u ||e |+ce +ud ||e uec|.
8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 248
£FI0£MI0I0C¥
Iocìdeoce Most common photodeimatosis.
Pievalence fiom 10% in Boston, 14% in Lon-
don, to 21% in Sweden. Aveiage age is 23 yeais,
much moie common in females. All iaces,
but most common in SPT I, II, III, and IV. In
Ameiican Indians (Noith and South Ameiica)
theie is a |ereJíìary type of PMLE that is called
atìínít ¡rurígo.
Ceo¿raphy PMLE is less fiequently obseived
in aieas that have high solai intensity thiough-
out the yeai and in peisons who have adapted
to peisistent sun exposuies. In fact, PMLE often
occuis foi the fiist time in peisons tiaveling foi
shoit vacations to tiopical aieas in wintei fiom
noithein latitudes.
FAIh0C£N£SIS
Possibly a delayed-type hypeisensitivity ie-
action to an (auto-) antigen induced by UVR;
suggested by the moiphology of the lesions and
the histologic pattein, which shows an infiltia-
tion of T cells. Moie commonly, UVA is the
action spectium, but PMLE lesions have been
evoked with UVB and with both UVA and UVB.
Since UVA is tiansmitted thiough window
glass, PMLE can be piecipitated while iiding
in a cai. Aieas of the skin habitually exposed
(face and neck) aie often spaied, despite seveie
involvement of the aims, tiunk, and legs.
CIINICAI MANIF£SIAII0N
0oset aod 0uratìoo oI Iesìoos PMLE appeais
in spiing oi eaily summei, and not infiequently
the eiuption does not iecui by the end of sum-
mei, suggesting a °haidening." PMLE most of-
ten appeais within houis of exposuie and, once
established, peisists foi 7 to 10 days, theieby
limiting the vacationei's subsequent time in the
|o|,ro|p|ouº ||ç|| e|up||ou (|\|E) |º + |e|r
||+| deºc|||eº + ç|oup o| |e|e|oçeueouº, |d|·
op+|||c, +cqu||ed, +cu|e |ecu||eu| e|up||ouº c|+|·
+c|e||/ed |, de|+,ed +|uo|r+| |e+c||ouº |o u\k.
\+u||eº|ed |, .+||ed |eº|ouº, |uc|ud|uç e|,||er+·
|ouº r+cu|eº, p+pu|eº, p|+queº, +ud .eº|c|eº.
noWe.e|, |u e+c| p+||eu| ||e e|up||ou |º couº|º·
|eu||, rouoro|p|ouº.
B, |+| ||e roº| ||equeu| ro|p|o|oç|c |,peº +|e
||e p+pu|+| +ud p+pu|o.eº|cu|+| e|up||ouº.
F0I¥M0kFh0üS IIChI £küFII0N |C|·9 . o92.12
°
|C|·¹0 . |5o.+
sun. Symptoms aie piuiitus (may piecede the
onset of the iash) and paiesthesia (tingling).
Skìo Iesìoos The papulai (Fig. 10-8) and
papulovesiculai types aie the most fiequent.
Less common aie plaques oi uiticaiial
plaques (Fig. 10-9). The lesions aie pink to
ied. In the individual patient, lesions aie
quite monomoiphous, i.e., eithei papulai
oi papulovesiculai oi uiticaiial plaques.
Recuiiences follow the oiiginal pattein.
DIstrIhutIvn The eiuption often spaies the
face and appeais most fiequently on the foie-
aims, V aiea of the neck, aims, and chest (Fig.
10-8). The lesions may also occui on the face
(Fig. 10-9), if theie has not been pievious ex-
posuie.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Edema of the epideimis,
spongiosis, vesicle foimation, and mild lique-
faction degeneiation of the basal layei. A dense
lymphocytic infiltiate is piesent in the deimis,
with occasional neutiophils. Theie is edema of
the papillaiy deimis and endothelial swelling.
ImmuooI|uoresceoce (0ìrect) Negative. ANA
negative. Theie is no leukopenia.
0IACN0SIS
The diagnosis is not difficult: delayed onset
of eiuption, chaiacteiistic moiphology, his-
topathologic changes that iule out lupus eiy-
thematosus, and the histoiy of disappeaiance
of the eiuption in days. In plaque-type PMLE,
a biopsy and immunofluoiescence studies aie
mandatoiy to iule out SLE (Fig. 10-9). P|oìo-
ìesìíng is done with both UVB and UVA. Test
sites aie exposed daily, staiting with 2 MEDs
of UVB and UVA, iespectively, foi 1 week to 10
days, using inciements of the UV dose. In 50%
of patients, a PMLE-like eiuption will occui
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 249
FICük£ 10-8 Fo|ymorphìc |ì¿ht eruptìoo C|uº|e|º o| cou||ueu|, e\||ere|, p|u||||c p+pu|eº ou ||e e\poºed
c|eº|, occu||ed |u +u A|+||+u r+u ||e d+, |o||oW|uç ||e |||º| ºuu e\poºu|e o| ||e ºe+ºou. I|e e|up||ou +|ºo
|u.o|.ed ||e +|rº, |u| ºp+|ed ||e |+ce +ud do|º+| |+udº.
FICük£ 10-9 Fo|ymorphìc |ì¿ht eruptìoo E|,||er+|ouº p|+queº |u ||e |+ce |o||oW|uç |||º| ºuu e\poºu|e o|
||e ºe+ºou. I|e |u||e|||, d|º||||u||ou |º .e|, º|r||+| |o ||+| o| |upuº e|,||er+|oºuº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 250
in the test sites, confiiming the diagnosis. The
eiuption in the test site mimicks the type of
PMLE in that paiticulai patient. This also helps
to deteimine whethei the action spectium is
UVB, UVA, oi both.
C0ükS£ AN0 Fk0CN0SIS
The couise is chionic and iecuiient and may,
in fact, become woise each season. Although
some patients may develop °toleiance" by the
end of the summei, the eiuption usually iecuis
the following spiing and/oi when the peison
tiavels to tiopical aieas in the wintei. Howevei,
spontaneous impiovement oi even cessation of
eiuptions occuis aftei yeais.
MANAC£M£NI
Freveotìoo Sunblocks, even the potent UVA-
UVB sunscieens, aie not always effective but
should be tiied fiist in eveiy patient.
Systemìc -Caiotene, 60 mg thiee times a day
foi 2 weeks, befoie going in the sun. Oial pied-
nisone 20 mg/day given 2 days befoie and 2 days
duiing exposuie is a good piophylaxis. Also,
intiamusculai tiiamcinolone acetonide, 40 mg,
will suppiess an eiuption when administeied a
few days befoie a tiip to a sunny iegion.
FüvA Fhotochemotherapy This is veiy effec-
tive when given in eaily spiing by inducing °tol-
eiance" foi the summei. PUVA tieatments have
to be given befoie the sunny season, have to be
iepeated each spiing, but aie usually not neces-
saiy foi moie than 3 oi 4 yeais. Narrow-|anJ
UVB (311 nm) is used with equal success.
uucorrou ºuu||ç||·|uduced W|e+||uç cou||ued
|o e\poºed |od, º||eº.
E|up||ou occu|º W||||u r|uu|eº o| e\poºu|e +ud
|eºo|.eº |u + |eW |ou|º. \e|, d|º+|||uç +ud ºore·
||reº |||e |||e+|eu|uç.
Ac||ou ºpec||ur |º u\B, u\A, +ud .|º|||e ||ç|| o|
+u, cor||u+||ou ||e|eo|. \oº| corrou|, u\A
(||ç. ¹0·¹0).
|º +u |rred|+|e |,pe | |,pe|ºeuº|||.||, |eºpouºe
|o cu|+ueouº +ud/o| c||cu|+||uç p|o|o+||e|çeuº.
I|e|+p,. ru|||p|e p|o|o||e|+p, ºeºº|ouº |u |oW
|u| |uc|e+º|uç doºeº ou ||e º+re d+, ('|uº|
|+|deu|uç¨), o|+| |rruuoºupp|eºº|.e +çeu|º o|
p|+ºr+p|e|eº|º.
||e.eu||ou. ºuu +.o|d+uce, ºuuºc|eeuº W||| ||ç|
p|o|ec||ou |+c|o|º +ç+|uº| +c||ou ºpec||ur.
S0IAk ükIICAkIA |C|·9 . 103.9
°
|C|·¹0 . |5o.!
\+||ouº W+.e|euç||º o| u\k +ud/o| .|º|||e ||ç||
c+u e||c|| o| +çç|+.+|e + uur|e| o| de|r+|oºeº.
|u ||eºe c+ºeº ||e e|up||ou |º |u.+||+||, º|r||+| |o
||+| o| ||e p||r+|, coud|||ou.
Au +|||e.|+|ed ||º| |º ç|.eu |u +|p|+|e||c+| o|de|
|u I+||e ¹0·1, |u| || º|ou|d |e erp|+º|/ed ||+|
+rouç ||eºe d|ºo|de|º '|E |º |, |+| ||e roº|
|rpo||+u|.
Fh0I0£XAC£k8AI£0 0£kMAI0S£S
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 251
IA8I£ 10-T 0iseases E\acerbated by U|travio|et |rradiation
Acue |e||+ç|+
A|op|c ec/er+ |erp||çuº |o||+ceuº (e|,||er+|oºuº)
C+|c|uo|d º,ud|ore |||,||+º|º |u||+ p||+||º
Cu|+ueouº I ce|| |,rp|or+ |ºo||+º|º
|+||e| d|ºe+ºe ke||cu|+|e e|,||er+|ouº ruc|uoº|º º,ud|ore
|e|r+|or,oº|||º koº+ce+
||ººer|u+|ed ºupe|||c|+| +c||u|c po|o|e|+|oº|º 'e|o|||e|c de|r+||||º
E|,||er+ ru||||o|re Iupus erythematosus
|+r|||+| |eu|çu c||ou|c perp||çuº I|+uº|eu| +c+u||o|,||c de|r+|oº|º
(n+||e,·n+||e, d|ºe+ºe) (C|o.e| d|ºe+ºe)
Ke|+|oº|º |o|||cu|+||º (|+||e| d|ºe+ºe) ne|peº |+||+||º
||c|eu p|+uuº
FICük£ 10-10 So|ar urtìcarìa, test sìtes I|e uppe| |oW o| ||e |erp|+|e |eº| º||eº We|e e\poºed |o |uc|e+º·
|uç doºeº o| u\B +ud |e.e+|ed ou|, e|,||er+ (||çu|eº |ud|c+|e rl/cr
2
+pp||ed). 2+ |ou|º |+|e| ||e |erp|+|e |eº|
º||eº |u ||e |oWe| |oW We|e e\poºed |o 0.5 +ud ¹ l/cr
2
u\A (W||c| +|e e\||ere|, |oW doºeº) +ud |rred|+|e|,
+||e| ||e e\poºu|e |||º p|c|u|e W+º |+|eu. |o|e r+ºº|.e u|||c+||+| |e+c||ou |u ||e u\A·e\poºed |eº| º||eº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 252
|o|p|,||+ cu|+ue+ |+|d+ (|CI) occu|º roº||, |u
+du||º.
|+||eu|º do uo| p|eºeu| W||| c|+|+c|e||º||c p|o·
|oºeuº|||.||, |u| W||| corp|+|u|º o| '||+ç||e º||u,¨
.eº|c|eº, +ud |u||+e, p+|||cu|+||, ou ||e do|º+ o|
||e |+udº, +||e| r|uo| ||+ur+.
I|e d|+çuoº|º |º cou|||red |, ||e p|eºeuce o| +
p|u||º|·|ed ||uo|eºceuce |u ||e u||ue W|eu e\+r·
|ued W||| + wood |+rp.
|CI |º d|º||uc| ||or .+||eç+|e po|p|,||+ (\|) +ud
+cu|e |u|e|r|||eu| po|p|,||+ (A||) |u ||+| p+||eu|º
W||| |CI do uo| |+.e +cu|e |||e·|||e+|eu|uç
+||+c|º.
|u|||e|ro|e, ||e d|uçº ||+| |uduce |CI +|e |eWe|
||+u ||e d|uçº ||+| |uduce \| +ud A||.
|o| c|+ºº|||c+||ou o| ||e po|p|,||+º, ºee I+||e
¹0·3.
F0kFh¥kIA CüIAN£A IAk0A |C|·9 . 211.¹
°
|C|·¹0 . E30.¹
M£IA80IIC Fh0I0S£NSIIIvII¥-Ih£ F0kFh¥kIAS
IA8I£ 10-8 C|assification and 0ifferentia| 0ia¿nosis of Porphyrias
0oogeo|ta| Porphyr|a |oterm|tteot
£rythropo|et|c £rythropo|et|c 00taoea Var|egate Ac0te
Porphyr|as Protoporphyr|a Tarda Porphyr|a Porphyr|a
|u|e|||+uce Au|oºor+| Au|oºor+| Au|oºor+| Au|oºor+| Au|oºor+|
|eceºº|.e dor|u+u| dor|u+u| dor|u+u| dor|u+u|
(|+r|||+| |o|r)
'|çuº +ud º,rp|orº
||o|oºeuº|||.||, \eº \eº \eº \eº |o
Cu|+ueouº |eº|ouº \eº \eº \eº \eº |o
A||+c|º o|
+|dor|u+| p+|u |o |o |o \eº \eº
|eu|opº,c||+|||c
º,ud|ore |o |o |o \eº \eº
|+|o|+|o|,
+|uo|r+||||eº + + + + +
ked ||ood ce||º
||uo|eºceuce + + - - -
u|opo|p|,||u +++ | | | |
Cop|opo|p|,||u ++ + | | |
||o|opo|p|,||u (+) +++ | | |
||+ºr+
||uo|eºceuce + + - + -
u||ue
||uo|eºceuce - - + ± -
|o|p|o||||uoçeu | | | (+++) (+++)
u|opo|p|,||u +++ | +++ +++ +++
|eceº
||o|opo|p|,||u + ++ | +++ |
|o|e. |, uo|r+|, +, +|o.e uo|r+|, ++, rode|+|e|, |uc|e+ºed, +++, r+||ed|, |uc|e+ºed, (+++), ||equeu||, |uc|e+ºed (depeudº ou W|e||e|
p+||eu| |+º +u +||+c|, o| |º |u |er|ºº|ou), (+), |uc|e+ºed |u ºore p+||eu|º.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 253
£FI0£MI0I0C¥
Onset 30 to 50 yeais, iaiely in childien; fe-
males on oial contiaceptives; males on estiogen
theiapy foi piostate cancei. Equal in males and
in females.
heredìty Most PCT patients have ìy¡e I ( at-
quíreJ ) induced by diugs oi chemicals. Ty¡e II
(|ereJíìary ), autosomal dominant; possibly
these patients actually have VP, but this is not
yet iesolved. Theie is also a °dual" type with VP
and PCT in the same family.
£II0I0C¥ AN0 FAIh0C£N£SIS
PCT is caused by eithei an inheiited oi acquiied
deficiency of UROGEN decaiboxylase. In type
I (spoiadic, acquiied PCT-symptomatic) the
enzyme is deficient only in the livei; in type
II (PCT-heieditaiy) it is also deficient in ied
blood cells (RBCs) and fibioblasts. C|emíta|s
anJ Jrugs ì|aì ínJute PCT. Ethanol, estiogen,
hexachloiobenzene (fungicide), chloiinated
phenols, iion, tetiachloiodibenzo- ¡ -dioxin.
High doses of chloioquine lead to clinical
manifestations in °latent" cases (low doses aie
used as tieatment). Oì|er ¡reJís¡osíng [atìors :
Diabetes mellitus (25%), hepatitis C viius, also,
hemochiomatosis.
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos No acute skin changes
but giadual onset. Patients may piesent with
fiagility of skin and bullae on the hands and
feet based on a photosensitivity ieaction to sun
and yet will have a suntan. Pain fiom eiosions in
easily tiaumatized skin (°fiagile skin").
Skìo Iesìoos Tense bullae and eiosions on
noimal-appeaiing skin (Fig. 10-11); slowly heal
to foim pink atiophic scais, milia (1-2 mm)
on doisa of hands and feet, nose, foiehead, oi
(bald) scalp. Puiple-ied suffusion (°heliotiope")
of cential facial skin (Fig. 10-12 À ), especially
peiioibital aieas. Biown hypeimelanosis, diffuse,
on exposed aieas. Hypeitiichosis of face (Fig.
10-13). Scleiodeima-like changes, diffuse oi
ciicumsciibed, waxy yellowish-white aieas on
exposed aieas of face (Fig. 10-12 B ), neck, and
tiunk, spaiing the doubly clothed aiea of the
bieast in females.
FICük£ 10-11 Forphyrìa cutaoea tarda Bu||+e +ud +||op||c dep|çreu|ed ºc+|º ou ||e do|ºur o| |o||
|+udº. I||º |º uo| +u +cu|e |e+c||ou |o |u|||+| ºuu e\poºu|e |u| de.e|opº o.e| ||re W||| |epe+|ed ºuu e\poºu|e
+ud occu|º +||e| r|uo| ||+ur+. I|e p+||eu| p|eºeu|º W||| + ||º|o|, o| ¨||+ç||e¨ º||u +ud |u||+e.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 254
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Bullae, subepideimal with
°festooned" (undulating) base. PAS staining
ieveals thickened vasculai walls. Paucity of an
inflammatoiy infiltiate.
ImmuooI|uoresceoce IgG and othei immu-
noglobulins at the deimal-epideimal junction
and in and aiound blood vessels, in the sun-
exposed aieas of the skin.
Chemìstry Plasma iion and livei enzymes may
be incieased. High level of iion stoies in the
livei. The patient may have hemochiomatosis.
B|ooJ g|utose is incieased in those patients with
diabetes mellitus (25% of patients).
Forphyrìo Studìes ìo Stoo| aod ürìoe
(Table 10-8) Incieased uiopoiphyiin (I iso-
mei, 60%) in uiine and plasma. Incieased
isocopiopoiphyiin (type III) and 7-caiboxyl-
poiphyiin in the feces. In contiast, VP has
maikedly elevated fecal piotopoiphyiin as the
diagnostic hallmaik. No inciease in -aminole-
vulinic acid oi poiphobilinogen in the uiine.
Sìmp|e Iest Wood lamp examination of the
uiine shows oiange-ied fluoiescence (Fig. 10-
14); to enhance, add a few diops of 10% hydio-
chloiic acid.
Iìver 8ìopsy Reveals poiphyiin fluoiescence
and often fatty livei. May also show ciiihosis,
hemochiomatosis.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
By clinical featuies, pink-ied fluoiescence of
uiine and elevated uiinaiy poiphyiins. Bullae
on doisa of hands and feet can occui in ¡seuJo-
PCT (see Section 22). Phototoxic ieactions oc-
cui in chionic ienal failuie with hemodialysis.
Tanning salon iadiation (visible and UVA). May
occasionally iesemble dyshidiotic eczema but
bullae aie on the doisa. E¡íJermo|ysís |u||osa
atquísíìa (see Section 6) has the same clinical
pictuie (incieased skin fiagility, easy biuising,
and light- and tiauma-piovoked bullae) and
some of the histology (subepideimal bullae
with little oi no deimal inflammation).
MANAC£M£NI
1. Avoid ethanol, stop diugs that could be
inducing PCT (such as estiogen), and elim-
inate exposuie to chemicals (chloiinated
phenols, tetiachloiodibenzo- ¡ -dioxin). In
some patients, complete avoidance of etha-
nol ingestion will iesult in a clinical and
biochemical iemission and in depletion of
the high level of iion stoies in the livei.
2. Phlebotomy is done by iemoving 500 mL of
blood at weekly oi biweekly inteivals until
the hemoglobin is decieased to 10 g. Clinical
FICük£ 10-12 Forphyrìa cutaoea tarda â. \e|, ºu|||e pe||o||||+| .|o|+ceouº co|o|+||ou.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 255
and biochemical iemission occuis within 5
to 12 months aftei iegulai phlebotomy. Re-
lapse within a yeai is uncommon (5-10%).
3. Low-dose chloioquine is used to induce
iemission of PCT in patients in whom
phlebotomy is contiaindicated because of
anemia. Since chloioquine can exaceibate
the disease and, in highei doses, may even
induce hepatic failuie in these patients, this
tieatment iequiies consideiable expeiience.
Howevei, long-lasting iemissions and, in a
poition of patients, clinical and biochemical
°cuie" can be achieved.
The best appioach cuiiently used by one of us
(K.W.) is to stait with a couise of thiee con-
secutive phlebotomies eveiy othei day followed
by 150 mg/d, of chloioquine PO. Close clini-
cal and laboiatoiy monitoiing (tiansaminases,
poiphyiin excietion in uiine), aie iequiied to
adjust the chloioquine dose, which is eventually
tapeied to 150 mg twice a week and continued
foi seveial months.
FICük£ 10-12 Forphyrìa cutaoea tarda (Cootìoued) 8. 'c|e|ode|ro|d |||c|eu|uç, ºc+|º, +ud e|oº|ouº
ou ||e |o|e|e+d.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 256
FICük£ 10-13 Forphyrìa cutaoea tarda n,pe||||c|oº|º |u + Wor+u W|o |+d |eeu ou + p|o|ouçed |eç|reu
W||| eº||oçeuº. uude| wood ||ç|| |e| u||ue º|oWed + |||ç|| co|+|·|ed ||uo|eºceuce, +º º|oWu |u ||ç. ¹0·¹+.
FICük£ 10-14 Forphyrìa cutaoea tarda: Wood |ì¿ht Co|+|·|ed ||uo|eºceuce o| ||e u||ue o| + p+||eu| W|||
|CI +º corp+|ed |o ||+| o| + uo|r+| cou||o|.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 25T
£FI0£MI0I0C¥
A¿e oI 0oset At pubeity; peak, second to
fouith decades.
kace All iaces; especially common in white
South Afiicans (3:1000) (a laige piopoition of
the piesent white population was descended
fiom an eaily Dutch settlei who emigiated to
South Afiica fiom Holland in 1680 to wheie VP
can be tiaced).
Iocìdeoce It is incieasingly iecognized in Eu-
iope (Finland) and the United States.
heredìty Autosomal dominant.
£II0I0C¥ AN0 FAIh0C£N£SIS
PROTOGEN oxidase defect iesulting in an
accumulation of piotophyiinogen in the livei,
which is excieted in the bile and is nonenzy-
matically conveited to piotopoiphyiin; this
accounts foi the high fecal piotopoiphyiin.
The basic metabolic defect is accentuated by
ingestion of ceitain diugs (Table 10-9), with
the iesultant piecipitation of acute attacks of
abdominal pain and neuiopsychiatiic disoideis
(deliiium, seizuies, peisonality changes).
\+||eç+|e po|p|,||+ (\|) |º + ºe||ouº +u|oºor+|
dor|u+u| d|ºo|de| o| |ere ||oº,u||eº|º.
'||u |eº|ouº ||+| +|e |deu||c+| |o ||oºe o| |CI
(.eº|c|eº +ud |u||+e, º||u ||+ç||||,, r|||+, +ud ºc+|·
||uç o| ||e do|º+ o| ||e |+udº +ud ||uçe|º).
Acu|e +||+c|º o| +|dor|u+| p+|u, ueu|opº,c||+|||c
r+u||eº|+||ouº.
|uc|e+ºed e\c|e||ou o| po|p|,||uº, eºpec|+||, c|+|·
+c|e||º||c +|e ||ç| |e.e|º o| p|o|opo|p|,||u |u ||e
|eceº.
',¤¤¤,¤ |o|p|,||+ .+||eç+|+.
*
|u 'ou|| A|||c+
vAkI£CAI£ F0kFh¥kIA |C|·9 . 211. ¹
°
|C|·¹0 . E30.2 ( )
CIINICAI MANIF£SIAII0N
Chao¿e wìth Seasoos Skin lesions occui duiing
the summei season but may peisist thioughout
the wintei; lesions iesult fiom exposuie to sun-
light. Painful eiosions, skin fiagility.
Systems kevìew Acute attacks of abdominal
pain, constipation, nausea and vomiting, muscle
weakness, seizuies, confusional state, psychiatiic
symptoms (depiession, coma); iaiely, cianial
neive involvement, bulbai paialysis, sensoiy
loss, and paiesthesias.
0ru¿ £xposure See Table 10-9.
Skìo Iesìoos PCT-like vesicles oi, moie
commonly, bullae (Fig. 10-15); eiosions, milia;
scleiosis (scleiodeima-like changes); scais
(pink, atiophic). Peiioibital heliotiope hue,
diffuse melanodeima and hypeitiichosis on
exposed aieas. Localization to doisa of hands,
fingeis, and feet, as in PCT.
Mìsce||aoeous Fìodìo¿s Neuiologic, especially
peiipheial neuiopathy.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y As foi PCT
IA8I£ 10-9 0ru¿s hatardous to Patients with Varie¿ate Porphyria
Aueº||e||cº. |+||||u|+|eº +ud |+|o||+ue |r|p|+r|ue
Au||cou.u|º+u|º. |,d+u|o|uº, \e||,|dop+
c+||+r+/ep|ue, e||oºu\|r|de, \|uo| ||+uqu|||/e|º. c||o|d|+/epo\|de,
re||ºu\|r|de, p|euºu\|r|de, p||r|doue d|+/ep+r, o\+/ep+r, ||u|+/ep+r,
rep|o|+r+|e
Au||r|c|o||+| +çeu|º. c||o|+rp|eu|co| ç||ºeo|u|.|u,
uo.o||oc|u, p,|+/|u+r|de, |eu|+/oc|ue
ºu||ou+r|deº ||eu,||u|+/oue
'u||ou,|u|e+º. c||o|p|op+r|de,
E|ço| p|ep+|+||ouº |o||u|+r|de
E||,| +|co|o| I|eop|,|||ue
no|roueº. eº||oçeuº, p|oçeº||u, o|+| cou||+cep||.e
p|ep+|+||ouº
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 258
Ceoera| Iaboratory £xamìoatìoo PvrphyrIns
See Table 10-8.
P|usmu Distinctive plasma fluoiescence with
emission maximum at 626 nm.
UrIne Incieased poiphobilinogen duiing
acute attacks.
Stvv| High piotopoiphyiin.
C0ükS£ AN0 Fk0CN0SIS
Lifetime disease. Piognosis good, if exaceibating
factois aie avoided. Raiely, death can occui aftei
ingestion oi injection of diugs (e.g., baibitu-
iates, geneial anesthesia) that induce incieased
amounts of cytochiome P450 and cieate a de-
mand foi incieased synthesis of heme.
0IFF£k£NIIAI 0IACN0SIS
Pseudopoiphyiia, scleiodeima, acquiied epidei-
molysis bullosa, heieditaiy copiopoiphyiia, PCT.
MANAC£M£NI
None; oial -caiotene may oi may not contiol
the skin manifestations but has no effect on
poiphyiin metabolism oi the impoitant sys-
temic manifestations.
FICük£ 10-15 varìe¿ate porphyrìa Bu||+e ou ||e do|ºur o| ||e |oo| +ud |oeº, + corrou º||e o| ºuu
e\poºu|e |u p+||eu|º We+||uç opeu |oo|We+|. I||º +2·,e+|·o|d |er+|e W+º d|+çuoºed W||| po|p|,||+ cu|+ue+ |+|d+.
I|e |eº|ouº |u po|p|,||+ cu|+ue+ |+|d+ +|e |deu||c+| |o ||e |eº|ouº |u .+||eç+|e po|p|,||+. I||º p+||eu|, |oWe.e|,
ç+.e + ||º|o|, o| |ecu||eu| +||+c|º o| +|dor|u+| p+|u, W||c| W+º + c|ue |o ||e d|+çuoº|º o| .+||eç+|e po|p|,||+,
|||º d|+çuoº|º W+º eº|+|||º|ed |, ||e de|ec||ou o| e|e.+|ed º|oo| p|o|opo|p|,||uº. \+||eç+|e po|p|,||+ (o| 'ou||
A|||c+u po|p|,||+) |º +||u |o +cu|e |u|e|r|||eu| po|p|,||+, |u W||c| ||e|e +|e uo º||u |eº|ouº |u| + |+|+| ou|core
r+, occu| W||| |uçeº||ou o| ce||+|u d|uçº (ºee I+||e ¹0·9). |u 'ou|| A|||c+ e.e|, W|||e p+||eu| W|o |º ºc|edu|ed
|o| r+jo| ºu|çe|, ruº| |+.e |+|o|+|o|, |eº|º |o| po|p|,||uº º|uce .+||eç+|e po|p|,||+ |º corrou |u ||+| couu||,.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 259
£FI0£MI0I0C¥
Iocìdeoce Uncommon; seiies iepoited fiom
Euiope (in The Netheilands, 1:100,000; Austiia,
United Kingdom) and the United States.
A¿e oI 0oset Acute photosensitivity begins
eaily in childhood; iaiely, late onset in eaily
adulthood.
Sex Equal in males and females.
kace All ethnic gioups, including blacks.
heredìty Autosomal dominant with vaiiable
penetiance.
FAIh0C£N£SIS
The defective enzyme is feiiochelatase. This
defect occuis at the step in poiphyiin metab-
olism in which piotopoiphyiin is conveited
to heme by feiiochelatase. This leads to an
accumulation of piotopoiphyiin that is highly
photosensitizing.
CIINICAI MANIF£SIAII0N
Impoitant sequence of symptoms: stinging,
buining, and itching occui wíì|ín a [ew mínuìes
of sunlight exposuie; eiythema and edema ap-
peai only aftei 1 to 8 h. Childien may choose
not to go out in the diiect sunlight aftei a
few painful episodes, which may cause seiious
sociopsychologic pioblems. Symptoms occui
when exposed to sunlight thiough window
glass. Photosensitivity is less common in the
wintei months in tempeiate aieas.
Systems kevìew Biliaiy colic, even in chil-
dien.
Skìo Chao¿es ìo Acute keactìoos to Suo|ì¿ht
£xposure Biight ied eiythema, latei edema
(swelling of hands especially), puipuia
¦especially on the nose, cheeks (Fig. 10-16),
I||º |e|ed||+|, re|+|o||c d|ºo|de| o| po|p|,||u
re|+|o||ºr |º uu|que +rouç ||e po|p|,||+º
|u ||+| po|p|,||uº o| po|p|,||u p|ecu|ºo|º +|e
uºu+||, uo| e\c|e|ed |u ||e u||ue.
E|,|||opo|e||c p|o|opo|p|,||+ (E||) |º c|+|+c|e|·
|/ed |, +u +cu|e ºuu|u|u·|||e p|o|oºeuº|||.||,, |u
cou||+º| |o ||e o||e| corrou po|p|,||+º (|CI o|
\|), |u W||c| o|.|ouº +cu|e p|o|oºeuº|||.||, |º ¤¤|
+ p|eºeu||uç corp|+|u|.
',rp|orº occu| |+p|d|, W||||u r|uu|eº o| ºuu
e\poºu|e +ud couº|º| o| º||uç|uç +ud |u|u|uç.
'||u º|çuº +|e e|,||er+, eder+, +ud pu|pu|+.
k+|e|, ||e|e r+, |e c||||oº|º o| ||e ||.e| +ud ||.e|
|+||u|e.
(||e.eu||.e) ||e+|reu| couº|º|º o| ·c+|o|eue
|0.
',¤¤¤,¤ E|,|||o|ep+||c p|o|opo|p|,||+.
£k¥Ihk0F0I£IIC Fk0I0F0kFh¥kIA |C|·9 . 211.¹
°
|C|·¹0 . |30.0
backs of hands (Fig. 10-17), and tips of eais].
Uiticaiia uncommon; vesicles oi bullae iaiely
occui. These changes appeai within 1-8 h and
aftei subjective symptoms and subside aftei
seveial houis oi days.
Skìo Chao¿es AIter Chrooìc kecurreot
£xposures
Shallow, often lineai scais, on the nose and
doisa of the hands (°aged knuckles"). Diffuse
wiinkling of the skin of the nose, aiound the
lips, and the cheeks, with obvious thickening
and a waxy coloi (Fig. 10-18). Ciusted, eiosive
lesions may occui on the nose and lips. In con-
tiast to PCT, absence of scleiodeimoid changes,
hypeitiichosis, and hypeipigmentation.
Ceoera| Medìca| Fìodìo¿s
Hemolytic anemia with hypeisplenism (iaie).
Cholelithiasis (12%), even in childien; stones
contain laige amounts of piotopoiphyiin. Livei
disease fiom massive deposition of piotopoi-
phyiin in hepatocytes occuis; fatal hepatic
ciiihosis is iaie, but occuis.
IA80kAI0k¥ £XAMINAII0NS
Forphyrìo Studìes (See Table 10-8) Incieased
piotopoiphyiin in ied blood cells, plasma, and
stools, but no excietion in the uiine except in
the iaie cases with fatal hepatic ciiihosis. De-
cieased activity of the enzyme feiiochelatase in
the bone maiiow, livei, and skin fibioblasts.
Iìver Fuoctìoo Tests foi livei function in-
dicated. Livei biopsy: poital and peiipoital
fibiosis and deposits of biown pigment and bi-
iefiingent gianules in hepatocytes and Kupffei
cells. Ciiihosis and poital hypeitension may
develop.
kadìo¿raphy Gallstones may be piesent.
Specìa| £xamìoatìoo Ior F|uoresceot £rythrocytes
RBCs in a blood smeai exhibit a chaiacteiistic
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 260
ìransíenì fluoiescence when examined with a
fluoiescence micioscope with a meicuiy oi
tungsten-iodide lamp that emits 400-nm iadi-
ation (Fig. 10-19).
0ermatopatho|o¿y Maiked eosinophilic ho-
mogenization and thickening of the blood vessels
in the papillaiy deimis; theie is an accumulation
of an amoiphous, hyaline-like eosinophilic sub-
stance in and aiound blood vessels.
0IACN0SIS
In EPP theie is photosensitivity with an exag-
geiated sunbuin iesponse without blisteis that
appeais much eailiei than oidinaiy sunbuin
eiythema. Also, the skin changes occui behind
window glass. T|ere ís no oì|er ¡|oìosensíìí·íìy
JísorJer ín w|ít| ì|e sym¡ìoms a¡¡ear so ra¡íJ|y
(mínuìes a[ìer ex¡osure ìo sun|íg|ì) . Poiphy-
iin examination establishes the diagnosis with
elevated fiee piotopoiphyiin levels in the RBCs
and in the stool. The fecal piotopoiphyiin is
most consistently elevated, but uiinaiy poiphy-
iins aie not. In chionic cases, the waxy thicken-
ing and wiinkling of facial skin is diagnostic.
0ìIIereotìa| 0ìa¿oosìs Hyalinosis cutis et
mucosae.
C0ükS£ AN0 Fk0CN0SIS
EPP peisists thioughout life, but the photosen-
sitivity may become less appaient in late adult-
hood. Livei ciiihosis may become manifest
in adults. Raiely, fatal outcome due to hepatic
failuie.
MANAC£M£NI
Theie is no tieatment foi the basic metabolic
abnoimality, but symptomatic ielief of the
photosensitivity can be achieved in most pa-
tients with oial -caiotene in divided doses of
180 mg/d. Theiapeutic levels of caiotenoids aie
achieved in 1-2 months. Patients on -caiotene
can iemain outdoois longei by a factoi of 8
to 10 but will still buin if exposuies aie too
long. Neveitheless, many patients can paitici-
pate in outdooi activities foi the fiist time.
Theie is no toxicity with piolonged tieatment
with -caiotene. Piotection by -caiotene can
be consideiably enhanced by PUVA-induced
tanning.
FICük£ 10-16 £rythropoì-
etìc protoporphyrìa ||||uºe
e|,||er+|ouº ºWe|||uç o| ||e
uoºe, |o|e|e+d, +ud c|ee|º
W||| pe|ec||+| |ero|||+çe +ud
|e|+uç|ec|+º|+. I|e|e +|e uo po|·
p|,||uº |u ||e u||ue. A c|ue |o ||e
d|+çuoº|º |º ||e ||º|o|, o| ||uç||uç
+ud |u|u|uç W||||u + |o 5 r|u
o| ºuu e\poºu|e. I|e |+ce o| |||º
Wor+u +ppe+|º ,e||oW·o|+uçe
|ec+uºe º|e W+º ou ·c+|o|eue,
W||c| o|.|ouº|, d|d uo| p|o|ec|
|e| ºu|||c|eu||,.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 261
FICük£ 10-1T £rythropoìetìc protoporphyrìa \+ºº|.e pe|ec||+|, cou||ueu| |ero|||+çe ou ||e do|º+ o|
||e |+udº o| + ¹o·,e+|·o|d 2+ | +||e| e\poºu|e |o ||e ºuu.
FICük£ 10-18 £rythropoìetìc protoporphyrìa
E|,||er+, eder+, e|oº|ou, c|uº||uç o| ||e uoºe W||| |eºº
ºe.e|e c|+uçeº ou ||e c||u o| + ¹5·,e+|·o|d |er+|e. |eep
W||u|||uç +ud + pecu||+| W+\, |||c|eu|uç ou ||e uppe| ||p
+ud c|ee|º r+|e ||e p+||eu| |oo| ruc| o|de| +º ||e, +|e
º|r||+| |o de|r+|o|e||oº|º |u p|o|o+çed º||u.
FICük£ 10-19 £rythropoìetìc protoporphyrìa
||uo|eºceu| |ed ||ood ce||º. I|e |e|| p+ue| º|oWº ||e
||ood ce|| ºre+| .|eWed W||| .|º|||e ||ç||. I|e ||ç||
p+ue| W+º ||e º+re ºre+| e\+r|ued W||| + |uuçº|eu·
|od|de |+rp ||+| er||º ou|, +00·ur |+d|+||ou. |o|e
|ed ||uo|eºceuce o| e|,|||oc,|eº. |o|e +|ºo ||+| ou|, +
r|uo|||, o| |ed ||ood ce||º ||uo|eºce +ud ||+| |o d|||e|eu|
deç|eeº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 262
kepe+|ed ºo|+| |uju||eº o.e| r+u, ,e+|º u|||r+|e|,
c+u |eºu|| |u ||e de.e|opreu| o| + º||u º,ud|ore,
!-·¤c|¤|-|·¤º·º (|ne).
|| occu|º |u pe|ºouº W||| '|I | |o ||| +ud |u pe|ºouº
W||| '|I |\ W|o |+.e |+d |e+., curu|+||.e e\po·
ºu|e |o ºuu||ç||, ºuc| +º |||eçu+|dº +ud ou|doo|
Wo||e|º, o.e| + |||e||re.
|ne deºc|||eº + po|,ro|p||c |eºpouºe o| .+||ouº
corpoueu|º o| ||e º||u (eºpec|+||, ce||º |u ||e
ep|de|r|º, ||e .+ºcu|+| º,º|er, +ud ||e de|r+|
couuec||.e ||ººue) |o p|o|ouçed +ud/o| e\ceºº|.e
ºuu e\poºu|e.
||º ºe.e|||, depeudº p||uc|p+||, ou ||e du|+||ou
+ud |u|euº||, o| ºuu e\poºu|e +ud ou ||e |ud|çeu·
ouº (couº|||u||.e) º||u co|o| +ud ||e c+p+c||, |o
|+u (|+cu||+||.e re|+u|u p|çreu|+||ou).
 || ,ou W+u| |o derouº||+|e |o +u o|de|
p+||eu| ||e |o|e o| u\k |u p|o|o+ç|uç juº| |+.e
||r/|e| uud|eºº +ud corp+|e ||e qu+|||, o|
||º/|e| |+c|+| º||u |o ||+| o| ||e ºup|+pu||c º||u.
0£kMAI0h£II0SIS ("Fh0I0ACINC") |C|·9 . o92.1+
°
|C|·¹0 . |51.9
Chk0NIC Fh0I00AMAC£
£FI0£MI0I0C¥
A¿e oI 0oset Most often in peisons >40
yeais.
Sex Highei incidence in males.
Skìo Fhototype Peisons with SPT I and II aie
most susceptible, but peisons with SPT III and
IV and even V (biown skin coloi) can develop
DHe.
Iocìdeoce Veiy common. The most susceptible
peisons with SPT I and II compiise about 25%
of the white population in the United States.
0ccupatìoo Faimeis (°faimei`s skin"), tel-
ephone linemen; sea woikeis (°sailoi`s skin"),
constiuction woikeis, and lifeguaids; ten-
nis, swimming, and ski instiuctois; moun-
tain guides, spoitspeisons, and °beach bums";
peisons who spend consideiable time in moun-
tain oi sea iesoits.
Ceo¿raphy DHe is moie seveie in white popu-
lations living in aieas with high solai UVR (at
high altitudes oi in low latitudes). Young white
childien (age 10) living in southein Boineo
(cool climate with high UVR) have been ob-
seived to have DHe, including solai keiatoses.
FAIh0C£N£SIS
While UVB is the most obvious damaging
UVR, UVA in high doses can pioduce connec-
tive tissue changes in mice. In addition, visible
(400-700 nm) and infiaied (1000-1,000,000
nm) iadiations have been implicated. The ac-
tion spectium foi DHe is not known foi ceitain;
theie is some expeiimental evidence in mice
that infiaied iadiation is implicated, in addition
to UVB and UVA.
CIINICAI MANIF£SIAII0N
Fersooa| hìstory Theie is a histoiy of in-
tensive exposuie to sun in youth (<20 yeais),
even though sun exposuie may have been quite
limited in latei adult life, and/oi significant sun
exposuie in adulthood. Because skin photo-
types aie genetically deteimined, theie is often
a family histoiy of DHe.
Skìo Iesìoos A combination of atiophy
(of epideimis), hypeitiophy (of papillaiy
deimis due to elastosis), telangiectases, spotty
depigmentation and hypeipigmentation, and
spotty hypeikeiatosis in light-exposed aieas.
Skin appeais wiinkled, wizened, leatheiy,
°piematuiely aged" (Fig. 10-20). Both
fine, cigaiette papei-like and deep fuiiow-
like wiinkling; skin is waxy, papulai with a
yellowish hue, and both glistening and iough
(Fig. 10-21). Theie may be telangiectasia
and biuising, Bateman oi senile puipuia
due to fiagility of small vessels. Maculai
hypeipigmentations: so|ar |enìígínes (see
below); maculai hypopigmentations; guììaìe
|y¡ome|anosís , <3 mm in diametei, on
the extiemities. Comedones, paiticulaily
peiioibital (teimed Fa·re-Ratout|eì Jísease ),
paiticulaily in cigaiette smokeis. Individuals
with DHe invaiiably have actinic keiatoses.
Also, seboiiheic keiatoses that aie misdiagnosed
as lentigos.
DIstrIhutIvn Exposed aieas, paiticulaily
face, peiioibital and peiioial aieas, scalp (bald
males). Nuchal aiea: cutis ihomboidalis (°ied
neck") with ihomboidal fuiiows; lowei aims,
doisa of hands. Pattein haii loss in both sexes,
although much less so in females.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 263
FICük£ 10-20 0ermatohe|ìosìs 'e.e|e deep W||u|||uç. I|e º||u +ppe+|º W+\,, p+pu|+| W||| + ,e||oW|º|
|ue (+c||u|c e|+º|oº|º). I||º o3·,e+|·o|d |er+|e rouu|+|u |+|re| ||.ed +| +u +||||ude o| ¹000 r +ud |+d |eeu
Wo|||uç ou|doo|º +|| |e| |||e. I|e|e |º |+º+| ce|| c+|c|uor+ |u ||e |e|| /,çor+||c |eç|ou.
FICük£ 10-21 Severe dermatohe|ìosìs oo the Iorearm oI a T0-year-o|d Iema|e Iarmhaod I|e º||u
|º W+\,, deep|, W||u||ed, +ud d|,. \u|||p|e ºo|+| |e|+|oºeº |+.e |eeu |ero.ed ||or |||º +|r |, c|,o||e|+p,.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 264
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Acanthosis of epideimis,
incieased hoiny layei. Flattening of the deimal-
epideimal junction. Atypia of the keiatinocytes.
Loss of small vessels in the papillaiy deimis.
E|asìosís : Degiaded elastic tissue with
accumulation of coaise amoiphous masses and
inciease in glycosaminoglycans in the uppei
deimis. Deciease in collagen.
C0ükS£ AN0 Fk0CN0SIS
The appeaiance of DHe maiks a ielatively
young peison as °old," a state that eveiyone tiies
to delay. DHe is inexoiably piogiessive and ii-
ieveisible, but some iepaii of connective tissue
effects can occui if the skin is piotected. Some
piocesses leading to DHe continue to piogiess,
howevei, even when sun exposuies aie seveiely
iestiicted in latei life; solai keiatoses and len-
tigines develop in the sun-damaged skin that
is now being piotected by avoidance and sun-
blocks. Yet theie aie documented examples of
spontaneous ieveisal of solai keiatoses.
MANAC£M£NI
Cuiient management is to pievent skin canceis
and the development of DHe with the use of
piotective sunblocks, a change of behavioi in
the sun, and the use of topical chemotheiapy
(tietinoin) that ieveises some of the changes
of DHe.
Iopìca| Ireatmeot Treìínoín in lotions, gels,
and cieams in vaiying concentiations ieveises
some aspects of DHe, especially in the connec-
tive tissue and vasculai changes. Topical ìa:aro-
ìene has also been shown to ieduce the effects
of photoaging in shoit-teim studies. Topical
tietinoin can altei the piogiession of incipient
epithelial skin canceis. 5-F|uorouratí| in lotions
and cieams and imiquimod aie highly effective
in causing a disappeaiance of solai keiatoses.
Topical imiquimod impioves cosmetic appeai-
ance of photoaged skin: histology shows iepaii
of photodamage.
Freveotìoo Peisons of SPT I and II should be
identified eaily in life and advised that they aie
susceptible to the development of DHe and skin
canceis, including melanoma. These peisons
should nevei sunbathe and should, fiom an eaily
age, adopt a daily piogiam of self-piotection
using sun-filteiing clothing and substantive and
effective topical sun-piotective solutions, gels,
oi lotions that can filtei DNA-damaging UVB;
effective UVA filteis aie now available. SPT I
and II peisons should avoid the peak houis of
UVB intensity, which aie the 2 h befoie and
aftei solai noon.
Cauìíon : Theie is some expeiimental evidence
that while sunscieens piotect fiom sunbuin,
they do not piotect fiom UV-induced local im-
munosuppiession. Pievention of sunbuin may
luie individuals into exposing themselves to the
sun foi piolonged peiiods, which may abiogate
immunosuiveillance mechanisms in the skin.
This has been linked to the iising incidence of
melanoma but is not pioven.
'o|+| |eu||ço |º + c||curºc|||ed ¹· |o !·cr
||oWu r+cu|e |eºu|||uç ||or + |oc+||/ed p|o|||·
e|+||ou o| re|+uoc,|eº due |o +cu|e o| c||ou|c
e\poºu|e |o ºuu||ç||.
\u|||p|e |eº|ouº uºu+||, +||ºe |u ºuu·e\poºed
º||eº.
S0IAk I£NIIC0 |C|·9 . 109.09
£FI0£MI0I0C¥ AN0 £II0I0C¥
A¿e oI 0oset Usually >40 yeais but may be
30 yeais in sunny climates and in susceptible
peisons.
kace Most common in Caucasians but seen
also in Asians.
Skìo Fhototype Geneially coiielated with skin
phototypes I to III and duiation and intensity
of solai exposuie.
£tìo|o¿y Solai lentigines may aiise acutely
aftei sunbuins and aftei oveidosage of PUVA
(PUVÀ |enìígínes ).
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 265
CIINICAI MANIF£SIAII0N
Skìo Iesìoos Stiictly maculai, 1 to 3 cm, and
as laige as 5 cm. Light yellow, light biown,
oi daik biown; vaiiegated mix of biown and
not unifoim coloi (Figs. 10-22 and 10-23),
as in café au lait macules. Round, oval, with
slightly iiiegulai boidei, ill defined (Fig. 10-23).
Scatteied, disciete lesions, stellate and shaiply
defined aftei acute sunbuin (Fig. 10-22).
DIstrIhutIvn Exclusively exposed aieas: foie-
head, cheeks, nose, doisa of hands and foie-
aims, uppei back, chest, shins.
FICük£ 10-22 0ermatohe|ìosìs: so|ar |eotì¿ìoes \u|||p|e º|e||+|e ||oWu r+cu|eº ou ||e º|ou|de|
occu||ed +||e| + ºuu|u|u. I|e, +|e +|| o| +|ou| ||e º+re º|/e +ud º|+|p|, r+|ç|u+|ed, W||c| |º c|+|+c|e||º||c
o| ºuu|u|u·|uduced ºo|+| |eu||ç|ueº.
FICük£ 10-23 0ermatohe|ìosìs: so|ar
|eotì¿ìoes \u|||p|e, .+||eç+|ed, |+u·|o·d+||·
||oWu r+cu|eº ou ||e r+|+| +ud ||ou|+| +|e+º
|u ||e |+ce. 'o|+| |eu||ç|ueº +|e uo| ||e º+re
+º ep|e||deº (||ec||eº)-||e, do uo| |+de |u
||e W|u|e| +º ||ec||eº do. |u cou||+º| |o ||e
º|+|p|, r+|ç|u+|ed ºo|+| |eu||ç|ueº º|oWu |u
||ç. ¹0·22, W||c| +|e due |o +u +cu|e ºuu|u|u,
||e ºo|+| |eu||ç|ueº º|oWu |e|e +|e o| d|||e|eu|
º|/eº +ud p+|||+||, ||| de||ued +ud cou||ueu|,
W||c| |º c|+|+c|e||º||c o| c||ou|c curu|+||.e
ºo|+| d+r+çe.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 266
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Club-shaped elongated
iete iidges that show hypeimelanosis and an
incieased numbei of melanocytes in the basal
layei.
0IFF£k£NIIAI 0IACN0SIS
8rowo Macu|es °Flat," acquiied, biown lesions
on the exposed skin of the face, which may on
cuisoiy examination appeai to be similai, have
distinctive featuies: solai lentigo, fieckles, seb-
oiiheic keiatosis, spieading pigmented actinic
keiatosis (SPAK), lentigo maligna.
MANAC£M£NI
Ciyosuigeiy oi lasei suigeiy aie effective. No
moie than 10 s of liquid nitiogen should
be administeied; otheiwise depigmentation of
noimal skin will occui.
uºu+||, occu|º +º + º|uç|e e|ouç+|ed, e\qu|º||e|,
|eude| uodu|e o| + "|e+d|uç" o| ||e ||ee |o|de|
o| |e||\ o| ||e e+|. Corrou, p|o|+||, due |o
couº|+u| rec|+u|c+| ||+ur+ +ud roº| p|o|+||,
|o u\ |+d|+||ou.
Appe+|º ºpou|+ueouº|,, eu|+|çeº qu|c||,, re+ºu|·
|uç |eºº ||+u ¹ cr (||ç. ¹0·2+), |||r, We||·de||ued,
|ouud |o o.+| W||| º|op|uç r+|ç|uº.
E|||e| er|edded |u ||e º||u o| e|e.+|ed ºe.e|+|
r||||re|e|º +ud W||| dore·º|+ped ºu||+ce, W|||e·
W+\, +ud ||+uº|uceu|, +ud o||eu u|ce|+|ed (||ç.
¹0·2+).
\o|e corrou |u r+|eº ||+u |u |er+|eº.
'pou|+ueouº p+|u o| |eude|ueºº |º ||e |u|||+| p|e·
ºeu||uç corp|+|u|. C+u |e |u|euºe +ud º|+|||uç,
p+|o\,ºr+| o| cou||uuouº.
||||e|eu||+| d|+çuoº|º |uc|udeº |+º+| ce|| +ud
ºqu+rouº ce|| c+|c|uor+, +c||u|c |e|+|oº|º, |u º||u
o| |u.+º|.e 'CC, |,pe|||op||c ºo|+| |e|+|oº|º, +ud
|e|+|o+c+u||or+. 0ue +|ºo |+º |o |||u| o| çou|,
|op|uº, ||eur+|o|d +ud ||eur+||c uodu|eº, +ud
d|ºco|d |upuº e|,||er+|oºuº.
\+u+çereu| |uc|udeº |u||+|eº|ou+| |ujec||ou o|
|||+rc|uo|oue +ce|ou|de, c+||ou d|o\|de |+ºe|,
ºu|çe|,. I|e de||u|||.e ||e+|reu| |º e\c|º|ou+|
ºu|çe|, |uc|ud|uç ||e uude||,|uç c+||||+çe.
Ch0N0k00£kMAIIIIS N00üIAkIS h£IICIS |C|·9 . !30.0
°
|C|·¹0 . no¹.0
FICük£ 10-24 Choodroder-
matìtìs oodu|arìs he|ìcìs Au
e\||ere|, p+|u|u| uodu|e W|||
ceu||+| u|ce|+||ou ou ||e +u||e||\ o|
+ o0·,e+|·o|d |er+|e. I|e ceu||+|
u|ce| |º co.e|ed W||| + c|uº| +ud
c+u |e r|º|+|eu |o| + |+º+| ce||
c+|c|uor+.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 26T
I|eºe º|uç|e o| ru|||p|e, d|ºc|e|e, d|,, |ouç|, +d·
|e|eu| ºc+|, |eº|ouº occu| ou ||e |+|||u+||, ºuu·
e\poºed º||u o| +du||º, uºu+||, ou + |+c|ç|ouud
o| de|r+|o|e||oº|º.
Ac||u|c |e|+|oºeº c+u p|oç|eºº |o ºqu+rouº ce||
c+|c|uor+.
',¤¤¤,¤ . 'o|+| |e|+|oº|º.
ACIINIC k£kAI0SIS |C|·9 . 102.0
°
|C|·¹0 . |51.0
£FI0£MI0I0C¥
A¿e oI 0oset Middle age, although in Aus-
tialia and southwestein United States solai
keiatoses may occui in peisons <30 yeais.
Sex Moie common in males.
kace SPT I, II, and III; iaie in SPT IV; almost
nevei in blacks oi South Indians.
0ccupatìoo Outdooi woikeis (especially faim-
eis, iancheis, sailois) and outdooi spoitspei-
sons (tennis, golf, mountain climbing, deep-sea
fishing).
FAIh0C£N£SIS
Piolonged and iepeated solai exposuie in sus-
ceptible peisons (SPT I, II, and III) leads to
cumulative damage to keiatinocytes by the ac-
tion of UVR, piincipally, if not exclusively, UVB
(290-320 nm).
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos (Fig. 10-25) Months to yeais.
Skìo Symptoms Lesions may be tendei. On
examination, painful if excoiiated with a fingei-
nail; patient winces.
FICük£ 10-25 So|ar keratoses E|,||er+|ouº r+cu|eº +ud p+pu|eº W||| co+|ºe, +d|e|eu| ºc+|e |ecore
cou||ueu| ou |||º |+|d ºc+|p W||| de|r+|o|e||oº|º. I|eºe |,pe||e|+|oºeº +|e ,e||oW|º|·ç|e,. I|e, +|e |e||e| |e||
||+u ºeeu, çeu||, +||+d|uç |eº|ouº W||| + ||uçe|u+|| uºu+||, |uduceº p+|u, e.eu |u e+||, ºu|||e |eº|ouº, + |e|p|u|
d|+çuoº||c ||ud|uç.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 268
Skìo Iesìoos Adheient hypeikeiatotic scale,
which is iemoved with difficulty and pain (Figs.
10-25 and 10-26). May be papulai. Skin-coloied,
yellow-biown, oi biown-°diity" (Fig. 10-26);
often theie is a ieddish tinge (Fig. 10-25). Rough,
like coaise sandpapei, °bettei felt than seen" on
palpation with a fingei. Most commonly <1 cm,
oval oi iound (Fig. 10-27À, B).
S¡etía| Presenìaìíon : SPAK (spieading pig-
mented actinic keiatosis). This lesion is best
desciibed as °looks like lentigo maligna but
feels like actinic keiatosis" (Fig. 10-28). It is a
iathei uncommon vaiiant of solai keiatosis.
The distinctive featuies of SPAK include size
(>1.5 cm), pigmentation (biown to black and
vaiiegated), and histoiy of sopui spieading,
especially the veiiucous suiface. The lesion is
impoitant because it can mimic lentigo ma-
ligna (LM). Keiatotic natuie of the lesion can
best be evaluated when the lesion is slightly
fiozen with LN2. Shaiply maiginated scale
is seen with solai and seboiiheic keiatoses.
Lentigos aie completely flat. It is, howevei,
easily distinguished fiom LM because LM is
completely flat without evidence of veiiucous
change. Biopsy is necessaiy to confiim the
clinical diagnosis.
DIstrIhutIvn Isolated single lesion oi scatteied
disciete lesions. Face ¦foiehead, nose, cheeks
(Figs. 10-25 and 10-26), temples, veimilion
boidei of lowei lip], eais (in males), neck
(sides), foieaims, and hands (doisa), shins, and
the scalp in bald males (Fig. 10-25). Males with
eaily pattein alopecia aie especially pione to
seveie deimatoheliosis and solai keiatosis on
the exposed scalp.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Laige biight-staining ke-
iatinocytes, with mild to modeiate pleomoi-
phism in the basal layei extending into follicles,
atypical (dyskeiatotic) keiatinocytes, paiakeia-
tosis.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Usually made on clinical findings. Diffeien-
tial: Chionic cutaneous lupus eiythematosus;
seboiiheic keiatosis, flat waits, squamous cell
caicinoma (SCC) in situ, supeificial basal cell
caicinoma. Highly hypeikeiatotic lesions and
SPAK may iequiie biopsy to iule out SCC (in
situ oi invasive) oi LM.
C0ükS£ AN0 Fk0CN0SIS
Solai keiatoses may disappeai spontaneously, but
in geneial iemain foi yeais. The actual incidence
of SCC aiising in pieexisting solai keiatoses is
unknown but has been estimated at one SCC
developing annually in 1000 solai keiatoses.
MANAC£M£NI
Freveotìoo Avoided by use of highly effective
UVB/UVA sunscieens, which should be applied
daily to the face, neck, and eais duiing the sum-
mei in noithein latitudes foi SPT I and SPT
II peisons and foi those SPT III peisons who
sustain piolonged sunlight exposuies.
Iopìca| Iherapy Cryvsurgery Light spiay oi
with cotton-tipped applicatoi is effective in
most cases.
3-F|uvrvurucI| (3-FU) Creum 3% Effective,
but difficult foi many individuals. Tieatment
of facial lesions causes significant eiythema
and eiosions, iesulting in tempoiaiy cosmetic
disfiguiement. Apply twice daily foi 2 to 4 weeks
on face; may iequiie longei peiiod of theiapy
on doisum of hands oi lowei legs. Efficacy can
be incieased and duiation of tieatment can be
shoitened if applied undei occlusion and/oi
combined with topical tietinoin. This, howevei,
leads to confluent eiosions and may iequiie
hospitalization. Reepithelialization occuis aftei
tieatment is discontinued. Pietieatment with
light ciyosuigeiy to hypeikeiatotic lesions may
impiove efficacy of 5-FU cieam.
1mIquImvd (twIce wee||y ]vr 16 wee|s)
Causes cytokine deimatitis, also leads to iiiita-
tion and eiosions but is highly effective.
TvpIcu| RetInvIds Used chionically, is effec-
tive foi tieatment of deimatoheliosis and supei-
ficial solai keiatoses.
DIc|v]enuc Ge| Used chionically, is effective
in supeificial acting keiatoses; also iiiitating.
FucIu| Pee|s Tiichloioacetic acid (5-10%) ef-
fective foi widespiead lesions.
Luser Surgery Eibium oi caibon dioxide la-
seis. Usually effective foi individual lesions.
Foi extensive facial lesions, facial iesuifacing
is effective.
PhvtvdynumIc Therupy Effective but painful
and cumbeisome.
Systemìc Iherapy Acitietin oi isotietinoin aie
effective in ieducing the numbei of solai keia-
toses and SCC in situ in patients with advanced
deimatoheliosis, especially in immunocompio-
mised patients. Lesions iecui once theiapy is
discontinued.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 269
FICük£ 10-26 Actìoìc keratoses, c|ose up \e||oW·ç|e,|º| ||ç|||, +d|e|eu| ºc+|eº ou ||e |o|e|e+d o| +u
30·,e+|·o|d r+u. A||+d|uç ||eºe |,pe||e|+|oºeº |º p+|u|u|. I|e|e |º + ºr+|| |+º+| ce|| c+|c|uor+ +| ||e |o|de| o|
||e |+||, ºc+|p.
FICük£ 10-2T So|ar keratoses, hì¿her ma¿oìIìcatìoo  A º|+|p|, de||ued ,e||oW·||oWu|º| ||ç|||,
+d|e|eu|, |ouç| |,pe||e|+|oº|º.  I||º |eº|ou |º e.eu ro|e e|e.+|ed +ud |+º + 'º|uc|·ou¨ +ppe+|+uce |||e + ºe|·
o|||e|c |e|+|oº|º. noWe.e|, || |º uo| ç|e+º, +ud ºo|| |u| |+||e| |+|d, |ouç|, +ud p+|u|u| W|eu ºc|+ped.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 2T0
SkIN k£ACII0NS I0 I0NIIINC kA0IAII0N
k+d|+||ou de|r+||||º |º de||ued +º º||u c|+uçeº
|eºu|||uç ||or e\poºu|e |o |ou|/|uç |+d|+||ou.
|-.-·º·||- -||-:|º +|e p+|u, e|,||er+, ep||+||ou,
ºupp|eºº|ou o| ºe|+ceouº ç|+udº, +ud p|çreu|+·
||ou (|+º||uç |o| Wee|º |o rou||º |o ,e+|º).
|··-.-·º·||- -||-:|º +|e +||op|,, ºc|e|oº|º,
|e|+uç|ec|+º|+º, u|ce|+||ou, +ud |+d|+||ou·|uduced
c+uce|º.
kA0IAII0N 0£kMAIIIIS |C|·9 . o92.32
°
|C|·¹0 . |53
Iype oI £xposure
Result of theiapy (foi cancei, foimeily also
used foi acne and psoiiasis, and fungal infec-
tions of the scalp in childien), accidental, oi
occupational (e.g., foimeily, in dentists who
held the film in the mouth with theii fingeis).
The iadiation causing iadiodeimatitis includes
supeificial and deep x-iay iadiation, election-
beam theiapy, and gienz-iay theiapy. It is a
pievailing myth among some deimatologists
that gienz iays aie °soft" and not caicinogenic;
it has been estimated that SCC can appeai fiom
>5000 cGy of gienz iays.
Iypes oI keactìoos
Acute Tempoiaiy eiythema that lasts 3 days
and then peisistent eiythema, which ieaches a
peak in 2 weeks and is painful; pigmentation
appeais about day 20; a late eiythema can also
occui beginning on day 35-40, and this lasts
2-3 weeks. Massive ieactions lead to blisteiing
eiosions (Fig. 10-29) and ulceiation, also pain-
ful; may occui as iecall phenomenon. Peima-
nent scaiiing may iesult.
Chrooìc Aftei [ratìíona| but ielatively intensive
theiapy with total doses of 3000-6000 iad, theie
develops an epideimolytic ieaction in 3 weeks.
This is iepaiied in 3-6 weeks, and scais and hy-
popigmentation develop; theie is loss of all skin
appendages and atiophy of the epideimis and
deimis. Duiing the next 2-5 yeais, the atiophy
incieases (Fig. 10-30); theie is hypei- and hy-
popigmentation (poikilodeima), telangiectasia
(Figs. 10-30 and 10-31), and supeificial venules
FICük£ 10-28 Spreadìo¿ pì¿meoted actìoìc keratosìs (SFAk) '|oo|º |||e |eu||ço r+||çu+¨ (ºee ||ç. ¹2·1) |u|
|º |ouç| +ud ||e|e|o|e '|ee|º |||e +c||u|c |e|+|oº|º.¨ A uoup|çreu|ed +c||u|c |e|+|oº|º |º ºeeu |u ||e p|e+u||cu|+| |eç|ou.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 2T1
become ectatic. Theie aie hypeikeiatoses (x-iay
keiatoses) (Fig. 10-34 À ). Neciosis and painful
ulceiation (Fig. 10-32) aie iaie but occui in ac-
cidental exposuie oi eiioi in dose: eithei one oi
a few accidentally high doses oi multiple small
doses at fiequent inteivals (monthly oi weekly).
When neciosis occuis, it is leatheiy, yellow, and
adheient and the base and suiiounding skin
aie extiemely painful (Fig. 10-32). Ulceiations
have a veiy pooi tendency to heal and usually
iequiie suigical inteivention. Accidental expo-
suie occuis mostly in occupational exposuie
and affects the hands, feet, and face. Theie is a
destiuction of the fingeipiint pattein, xeiosis,
scanty haii, atiophy of sebaceous and sweat
glands, and development of keiatoses (Fig.
10-34À ).
Naì|s Longitudinal stiiations (Fig. 10-34 B )
show thickening, dystiophy. Diclofenac used
chionically is effective in keiatoses.
C0ükS£, Fk0CN0SIS, AN0 MANAC£M£NI
Chionic iadiation deimatitis is peimanent,
piogiessive, and iiieveisible. SCC may develop
in 4-40 yeais (Figs. 10-33 and 10-34À , B),
with a median of 7-12 yeais, almost exclu-
sively fiom the chionic iepeated types of ex-
posuies. SCC always develops within the aiea
of iadiodeimatitis, (Fig. 10-34À, B). Tumois
metastasize in about 25%; despite extensive
suigeiy (excision, giafts, etc.), the piognosis is
pooi, and iecuiiences aie common. Basal cell
caicinoma (BCC) may also occui in chionic
iadiation deimatitis and appeais mostly in
patients foimeily tieated with x-iays foi acne
vulgaiis and acne cystica oi epilation (tinea
capitis) (Fig. 10-31). The tumois may ap-
peai 40-50 yeais aftei exposuie. Excision and
giafting aie often possible befoie the cancei
develops.
FICük£ 10-29 kadìatìoo dermatìtìs: acute, reca|| pheoomeooo I||º p+||eu| |+d ||e+º| c+uce|. '|e |+d
+ |urpec|or,, re||o||e\+|e, +ud \·|+, ||e|+p, +ud de.e|oped p+|u|u| e|,||er+ +ud e|oº|ouº +| ||e |||+d|+|ed º||e.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 2T2
FICük£ 10-30 kadìatìoo dermatìtìs: chrooìc I|e|e |º ºc|e|oº|º cor||ued W||| +||op|, +ud |e|+uç|ec|+º|+.
I||º |º ||e |eºu|| o| ||e |||+d|+||ou o| +u |u|+u|||e |er+uç|or+ |u |u|+uc,.
FICük£ 10-31 kadìatìoo dermatìtìs: chrooìc I|e|e |º po||||ode|r+ (||oWu. |,pe|p|çreu|+||ou, W|||e.
|,pop|çreu|+||ou, |ed. |e|+uç|ec|+º|+) cor||ued W||| +||op|, +ud ºc|e|oº|º. n+||º +|e +|ºeu|. I|eºe r+ºº|.e º||u
c|+uçeº +|e ||e |eºu|| o| o.e|doºed |||+d|+||ou ||e p+||eu| |ece|.ed +º + c|||d |o| |uuç+| |u|ec||ou o| ||e ºc+|p. ne
|º + c+ud|d+|e |o| 'CC |u ||e |u|u|e.
S£CII0N 10 |n0I0'E|'|I|\|I\, |n0I0·|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 2T3
FICük£ 10-32 kadìatìoo dermatìtìs: chrooìc Au
+|e+ o| ºe.e|e |e|+uç|ec|+º|+ W||| + ceu||+| uec|oº|º ||+|
|º |e+||e|,, ,e||oW|º|·W|||e, +ud ||ç|||, +d|e|eu|. 'u|ç|c+|
|ero.+| W||| |e.e+| + deep u|ce|. I|e |eº|ou |º e\||ere|,
p+|u|u|.
FICük£ 10-33 kadìatìoo dermatìtìs: chrooìc wìth
squamous ce|| carcìooma A |+|çe º||ç|||, e|e.+|ed
u|ce| |u +u +|e+ o| +||op|,, ||||oº|º, po||||ode|r+, +ud
|e|+uç|ec|+º|+ ou ||e c|eº| W+||. I||º occu||ed 20 ,e+|º
+||e| |+d|c+| r+º|ec|or,, +\|||+|, |,rp| uode d|ººec||ou,
+ud |+d|o||e|+p,. I|e u|ce|+||ou W+º p||r+|||, due |o
|+d|ouec|oº|º. |oW ||e |o|de| o| ||e u|ce| |º e|e.+|ed
+ud |||r. |||º |º 'CC +||º|uç |u |||º |+d|+||ou de|r+||||º.
FICük£ 10-34 kadìatìoo-ìoduced squamous ce|| carcìooma  I|eºe +|e ||e |+udº o| +u e|de||,
|+d|o|oç|º| W|o dec+deº +ço |+d d|º|eç+|ded p|ec+u||ou+|, re+ºu|eº +ud |+|d|, Wo|e ç|o.eº do|uç ||uo|oºcop|c
Wo||. I|e|e +|e ru|||p|e \·|+, |e|+|oºeº, ||e |,pe||e|+|o||c |eº|ou ou ||e ||ç|| ||ur| |+º deº||o,ed ||e u+|| +ud
|ep|eºeu|º \·|+,·|uduced 'CC.  |+|| c|+uçeº |u º||e o| |+d|+||ou e\poºu|e. |o|e ||e ||ue+| º|||+||ouº |eºu|||uç
||or d+r+çe |o ||e u+|| r+|||\. A| ||e u+|||o|d +ud e\|eud|uç p|o\|r+||, ou ||e ||ur|, ||e|e |º +u |||eçu|+| e|,·
||er+|ouº p|+que ||+| |ep|eºeu|º roº||, 'CC |u º||u |u|, |oc+||,, +|ºo |u.+º|.e 'CC.
â 8
8
2T4
S E C I | 0 N 1 1
Fk£CANC£k0üS I£SI0NS
AN0 CüIAN£0üS CAkCIN0MAS
Cu|+ueouº ep|||e||+| c+uce|º ¦uoure|+uor+
º||u c+uce| (|\'C)| +|e ||e e+º|eº| o| +||
c+uce|º |o d|+çuoºe +ud ||e+|. I|e, o||ç|u+|e
roº| corrou|, |u ||e ep|de|r+| çe|r|u+||.e
|e|+||uoc,|eº o| +due\+| º||uc|u|eº (e.ç., ºWe+|
+pp+|+|uº, |+|| |o|||c|e). I|e |Wo p||uc|p+|
|\'Cº +|e |+º+| ce|| c+|c|uor+ (BCC) +ud
ºqu+rouº ce|| c+|c|uor+ ('CC). 'CC o||eu
|+º ||º o||ç|u |u +u |deu||||+||e d,ºp|+º||c |u º||u
|eº|ou ||+| c+u |e ||e+|ed |e|o|e ||+u| |u.+º|ou
occu|º. |u cou||+º|, |u º||u BCC |º uo| |uoWu,
|u| r|u|r+||, |u.+º|.e 'ºupe|||c|+|¨ BCCº +|e
corrou.
I|e roº| corrou e||o|oç, o| |\'C |u
|+||·º||uued |ud|.|du+|º |º ºuu||ç||, u|||+.|o|e|
|+d|+||ou (u\k), +ud |ur+u p+p|||or+.||uº
(n|\). 'o|+| |e|+|oºeº +|e ||e roº| corrou
p|ecu|ºo| |eº|ouº o| 'CC |u º||u ('CC|') +ud
|u.+º|.e 'CC occu|||uç +| º||eº o| c||ou|c
ºuu e\poºu|e |u |ud|.|du+|º o| uo|||e|u Eu·
|ope+u |e|||+çe (ºee 'ec||ou ¹0). u\k +ud
n|\ c+uºe ||e ºpec||ur o| c|+uçeº |+uç|uç
||or ep|||e||+| d,ºp|+º|+ |o 'CC|' |o |u.+º|.e
'CC. \uc| |eºº corrou|,, |\'C c+u |e
c+uºed |, |ou|/|uç |+d|+||ou (+||º|uç |u º||eº
o| c||ou|c |+d|+||ou d+r+çe), c||ou|c |u||+r·
r+||ou, |,d|oc+||ouº (|+|), +ud c||ou|c |uçeº·
||ou o| |uo|ç+u|c +|ºeu|c, ||eºe |uro|º c+u |e
ruc| ro|e +çç|eºº|.e ||+u ||oºe +ººoc|+|ed
W||| u\k o| n|\. |u ||e |uc|e+º|uç popu|+·
||ou o| |rruuoºupp|eººed |ud|.|du+|º (||oºe
W||| n|\/A||' d|ºe+ºe, ºo||d o|ç+u ||+uºp|+u|
|ec|p|eu|º, e|c.), u\k· +ud n|\·|uduced 'CCº
+|e ruc| ro|e corrou +ud c+u |e ro|e
+çç|eºº|.e.
£FI0£kMAI Fk£CANC£kS AN0 CANC£kS
£P|Th£L|AL P8£0Ah0£800S L£S|0hS
Ah0 S00|S
Dysplasia of epideimal keiatinocytes in epi-
deimis and squamous mucosa can involve the
lowei poition of the epideimis oi the full
thickness. Basal cells matuie into dysplastic ke-
iatinocytes iesulting in a hypeikeiatotic papule,
oi plaque, clinically identified as °keiatosis."
A continuum exists fiom dysplasia to SCCIS
to invasive SCC. These lesions have vaiious
associated eponyms such as Bowen disease oi
eiythioplasia of Queyiat, which as desciiptive
moiphologic teims aie helpful; teims such as
UVR- oi HPV-associated SCCIS, howevei, will
be moie meaningful but can be used only foi
those lesions with known etiology.
Ep|||e||+| p|ec+uce|ouº |eº|ouº +ud 'CC|' c+u |e
c|+ºº|||ed +º |o||oWº.
u\k·|uduced
'o|+| (+c||u|c) |e|+|oºeº
'p|e+d|uç p|çreu|ed +c||u|c |e|+|oºeº
('|AK)
||c|euo|d +c||u|c |e|+|oºeº
BoWeuo|d +c||u|c |e|+|oºeº
'CC|' (BoWeu d|ºe+ºe)
n|\·|uduced
|oW·ç|+de ºqu+rouº |u||+ep|||e||+| |eº|ou
(|'||)
n|ç|·ç|+de ºqu+rouº |u||+ep|||e||+| |eº|ou
(n'||)
'CC|' (BoWeuo|d p+pu|oº|º)
A|ºeu|c+| |e|+|oºeº
|+|rop|+u|+| |e|+|oºeº
BoWeuo|d +|ºeu|c+| |e|+|oºeº
n,d|oc+||ou (|+|) |e|+|oºeº
I|e|r+| |e|+|oºeº
Ke|+|oºeº |u c||ou|c |+d|+||ou de|r+||||º
C||ou|c c|c+|||\ (ºc+|) |e|+|oºeº
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 2T5
S0LA8 08 A0T|h|0 k£8AT0S|S
These single oi multiple, disciete, diy, iough,
adheient scaly lesions occui on the habitually
sun-exposed skin of adults. They can piogiess
A cu|+ueouº |o|u (Cn) |º + :|·¤·:c| eu|||, |+.|uç
||e +ppe+|+uce o| +u +u|r+| |o|u W||| + p+pu|+|
o| uodu|+| |+ºe +ud + |e|+|o||c c+p o| .+||ouº
º|+peº +ud |euç||º (||ç. ¹¹·2).
Cnº roº| corrou|, |ep|eºeu| |,pe|||op||c ºo|+|
|e|+|oºeº. noWe.e|, |u º||u o| |u.+º|.e 'CC |º
o||eu p|eºeu| +| ||e |+ºe o| + Cn.
Cnº uºu+||, +||ºe W||||u +|e+º o| de|r+|o|e||oº|º
ou ||e |+ce, e+|, do|ºur o| |+udº, o| |o|e+|rº,
+ud º||uº.
|oup|ec+uce|ouº Cn |o|r+||ou c+u +|ºo occu| |u
ºe|o|||e|c |e|+|oºeº, W+||º, +ud |e|+|o+c+u||o·
r+º.
C||u|c+||,, Cnº .+|, |u º|/e ||or + |eW r||||re|e|º
|o ºe.e|+| ceu||re|e|º (||ç. ¹¹·2). I|e |o|u r+,
|e W|||e, ||+c|, o| ,e||oW|º| |u co|o| +ud º||+|ç||,
cu|.ed, o| ºp||+| |u º|+pe.
n|º|o|oç|c+||, ||e|e |º uºu+||, |,pe|||op||c +c||u|c
|e|+|oº|º, 'CC|' o| |u.+º|.e 'CC +| ||e |+ºe.
Bec+uºe o| ||e poºº||||||, o| |u.+º|.e 'CC, + Cn
º|ou|d +|W+,º |e e\c|ºed.
CüIAN£0üS h0kN |C|·9 . 102.2
°
|C|·¹0 . |35.3
to SCCIS, which can then piogiess to invasive
SCC. (Fig. 11-1).
Synonym : Solai and actinic keiatosis aie syn-
onymous.
Foi a full discussion of this condition, see Sec-
tion 10, p. 267.
FICük£ 11-1 So|ar keratoses aod ìovasìve squamous ce|| carcìooma \u|||p|e, ||ç|||, +d|e|eu| d|||,·
|oo||uç ºo|+| |e|+|oºeº (ºee +|ºo ||çº. ¹0·25 |o ¹0·21). I|e |+|çe uodu|e º|oWu |e|e |º co.e|ed |, |,pe||e|+|oºeº
+ud |ero|||+ç|c c|uº|º, || |º p+|||+||, e|oded +ud |||r. I||º uodu|e |º |u.+º|.e ºqu+rouº ce|| c+|c|uor+. I|e
|r+çe |º º|oWu |o derouº||+|e ||e ||+uº|||ou ||or p|ec+uce|ouº |eº|ouº |o ||+u| c+|c|uor+.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 2T6
Appe+| dec+deº +||e| c||ou|c +|ºeu|c |uçeº||ou
(red|c|u+|, occup+||ou+|, o| eu.||oureu|+| e\po·
ºu|e).
A|ºeu|c+| |e|+|oºeº |+.e ||e po|eu||+| |o |ecore
'CC|' o| |u.+º|.e 'CC. I|eºe +|e cu||eu||, |e|uç
ºeeu |u weº| Beuç+| +ud B+uç|+deº|.
IWo |,peº. puuc|+|e, ,e||oW p+pu|eº ou p+|rº
+ud ºo|eº (||ç. ¹¹·! 1 ), |e|+|oºeº |ud|º||uçu|º|+||e
||or +c||u|c |e|+|oºeº ou ||e ||uu| +ud e|ºe·
W|e|e. I|eºe +|e o||eu +ººoc|+|ed W||| ºr+||
'CC|' o| ||e BoWeu·|,pe +ud |,pop|çreu|ed
º||ç|||, dep|eººed r+cu|eº ('|+|ud|opº |u ||e
duº|¨) (||ç. ¹¹·! 3 ).
I|e+|reu|-+º |o| ºo|+| |e|+|oºeº.
AkS£NICAI k£kAI0S£S |C|·9 . o92.+
°
|C|·¹0 . |35.3
||eºeu|º +º ºo|||+|, o| ru|||p|e r+cu|eº, p+pu|eº,
o| p|+queº, W||c| r+, |e |,pe||e|+|o||c o|
ºc+||uç.
'CC|' |º roº| o||eu c+uºed |, u\k o| n|\
|u|ec||ou.
Corrou|, +||ºeº |u ep|||e||+| d,ºp|+º||c |eº|ouº
ºuc| +º ºo|+| |e|+|oºeº o| n|\·|uduced ºqu+·
rouº ep|||e||+| |eº|ouº ('||) (ºee 'ec||ouº 21
+ud !0).
||u| o| |ed, º|+|p|, de||ued ºc+|, p|+queº ou ||e
º||u +|e c+||ed 3¤+-¤ !·º-cº- , º|r||+| |u| uºu+||,
uouºc+|, |eº|ouº ou ||e ç|+uº +ud .u|.+ +|e c+||ed
-·,||·¤¤|cº·c .
Auoçeu||+| n|\·|uduced 'CC|' |º |e|e||ed |o +º
|¤+-¤¤·! ¤c¤±|¤º·º .
uu||e+|ed ''C|' r+, p|oç|eºº |o |u.+º|.e 'CC.
w||| n|\·|uduced 'CC|' |u n|\/A||', |eº|ouº
o||eu |eºo|.e corp|e|e|, W||| ºucceºº|u| AkI +ud
|rruue |ecouº|||u||ou.
I|e+|reu| |º |op|c+| 5·||uo|ou|+c||, |r|qu|rod,
c|,oºu|çe|,, C0
2
|+ºe| e.+po|+||ou, o| e\c|º|ou,
|uc|ud|uç \o|º r|c|oç|+p||c ºu|çe|,.
SÇüAM0üS C£II CAkCIN0MA IN SIIü |C|·9 . ¹1!.0
°
|C|·¹0 . \3010/2
FICük£ 11-2 Cutaoeous horo: hypertrophìc actìoìc keratosìs A |o|u|||e p|ojec||ou o| |e|+||u ou +
º||ç|||, |+|ºed |+ºe |u ||e ºe|||uç o| +d.+uced de|r+|o|e||oº|º ou ||e uppe| e,e||d |u +u 35·,e+|·o|d |er+|e. E\c|·
º|ou º|oWed |u.+º|.e 'CC +| ||e |+ºe o| ||e |eº|ou.
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 2TT
FICük£ 11-3 Arseoìca| keratoses  \u|||p|e puuc|+|e, ||ç|||, +d|e|eu| +ud .e|, |+|d |e|+|oºeº ou ||e
p+|r.  A|ºeu|c+| |e|+|oºeº ou ||e |+c|. \u|||p|e |eº|ouº +|e ºeeu |e|e |+uç|uç ||or |ed |o |+u, d+|| ||oWu, +ud
W|||e. I|e ||oWu |eº|ouº +|e + r|\ o| +|ºeu|c+| |e|+|oºeº (|+|d, |ouç|) +ud ºr+|| ºe|o|||e|c |e|+|oºeº (ºo|| +ud
ºroo||). I|e d|||e|euce c+u |e |e||e| |e|| ||+u ºeeu. I|e |ed |eº|ouº +|e ºr+|| BoWeuo|d |e|+|oºeº +ud BoWeu
d|ºe+ºe (ºee ||ç. ¹¹·+). I|e W|||e r+cu|+| +|e+º +|e º||ç|||, dep|eººed +ud |ep|eºeu| ºupe|||c|+| +||op||c ºc+|º
||or ºpou|+ueouº|, º|ed o| ||e+|ed +|ºeu|c+| |e|+|oºeº. I|e eu|||e p|c|u|e ç|.eº ||e |rp|eºº|ou o| '|+|u d|opº |u
||e duº|.¨


FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 2T8
£II0I0C¥
UVR, HPV, aisenic, tai, chionic heat exposuie,
chionic iadiation deimatitis.
CIINICAI MANIF£SIAII0N
Lesions aie most often asymptomatic but may
bleed. Nodule foimation oi onset of pain oi
tendeiness within SCCIS suggests piogiession
to invasive SCC.
Skìo Fìodìo¿s Appeais as a shaiply demaicated,
scaling, oi hypeikeiatotic macule, papule, oi
plaque (Fig. 11-4). Solitaiy oi multiple lesions
aie pink oi ied in coloi and have a slightly
scaling suiface, small eiosions, and can be
ciusted. Such lesions aie always well-defined
and aie called Bowen Jísease (Fig. 11-4).
Red, shaiply demaicated, glistening maculai
oi plaque-like SCCIS on the glans penis oi
labia minoia aie called eryì|ro¡|asía o[ Queyraì
(see Section 35). Anogenital HPV-induced SC-
CIS may be ied, tan, biown, oi black in coloi
and aie iefeiied to as |owenoíJ ¡a¡u|osís (see
Section 35). Eioded lesions may have aieas of
ciusting. SCCIS may be mistaken foi a patch
of eczema oi psoiiasis and go undiagnosed foi
yeais, iesulting in laige lesions with annulai oi
polycyclic boideis (Fig. 11-
5). Once invasion occuis,
nodulai lesions appeai
within the plaque and the
lesion is then commonly
called Bowen tartínoma
(Fig. 11-5).
DIstrIhutIvn UVR-in-
duced SCCIS commonly
aiises within a solai keia-
tosis in the setting of pho-
toaging (deimatoheliosis).
HPV-induced SCCIS aiises
within an aiea of low-giade
oi high-giade SIL, mostly in
the genital aiea but also pei-
iungually, most commonly
on the thumb oi in the nail
bed (see Fig. 33-15) (Image
11-1).
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Caicinoma in situ with
loss of epideimal aichitectuie and iegulai dif-
feientiation; keiatinocyte polymoiphism, single
cell dyskeiatosis, incieased mitotic iate, multi-
nucleai cells. Epideimis may be thickened but
basement membiane intact.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical diagnosis confiimed by deimatopatho-
logic findings. Diffeiential diagnosis includes all
well-demaicated pink-ied plaque(s): Nummu-
lai eczema, psoiiasis, seboiiheic keiatosis, solai
keiatoses, veiiuca vulgaiis, veiiuca plana, con-
dyloma acuminatum, supeificial BCC; amelan-
otic melanoma, Paget disease.
C0ükS£ AN0 Fk0CN0SIS
Untieated SCCIS will piogiess to invasive SCC
(Fig. 11-5). In HIV/AIDS, iesolves with suc-
cessful ART. Lymph node metastasis can occui
without demonstiable invasion. Metastatic dis-
semination fiom lymph nodes.
IMAC£ 11-1 Squamous ce||
carcìooma: p|ed||ec||ou º||eº.
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 2T9
MANAC£M£NI
Iopìca| Chemotherapy 5-F|uorouratí| cieam
applied eveiy day oi twice daily, with oi without
tape occlusion, is effective. So is ímíquímoJ, but
both iequiie consideiable time.
Cryosur¿ery Highly effective. Lesions aie usu-
ally tieated moie aggiessively than solai keia-
toses, and supeificial scaiiing will iesult.
Fhotodyoamìc Iherapy Effective but still cum-
beisome and painful.
Sur¿ìca| £xcìsìoo Has the highest cuie iate
but the gieatest chance of causing cosmetically
disfiguiing scais. It should be done in all lesions
wheie invasion cannot be excluded by biopsy.
FICük£ 11-4 Squamous ce|| carcìooma ìo sìtu: 8oweo dìsease  A |+|çe, º|+|p|, der+|c+|ed, ºc+|,,
e|,||er+|ouº p|+que º|ru|+||uç + pºo||+||c |eº|ou.  A º|r||+| pºo||+º||o|r p|+que W||| + r|\ o| ºc+|eº, |,pe|·
|e|+|oº|º, +ud |ero|||+ç|c c|uº|º ou ||e ºu||+ce.
 
FICük£ 11-5 Squamous ce|| carcìooma ìo sìtu: 8oweo dìsease aod ìovasìve SCC: 8oweo carcìooma
A |+|çe .+||eç+|ed o|+uçe, ||oWu |o ç|+, p|+que ou ||e |+c|, º|+|p|, de||ued, W||| |||eçu|+| ou|||ueº |ep|eºeu|º
'CC|', o| BoWeu d|ºe+ºe. I|e |ed uodu|e ou |||º p|+que |ud|c+|eº ||+| |e|e ||e |eº|ou |º uo| +u, ro|e +u |u º||u
|eº|ou |u| ||+| |u.+º|.e c+|c|uor+ |+º de.e|oped. || |º +u uud|||e|eu||+|ed c+|c|uor+.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 280
'CC o| ||e º||u |º + r+||çu+u| |uro| o| |e|+||uo·
c,|eº, +||º|uç |u ||e ep|de|r|º.
'CC uºu+||, +||ºeº |u ep|de|r+| p|ec+uce|ouº
|eº|ouº (ºee +|o.e) +ud, depeud|uç ou e||o|oç,
+ud |e.e| o| d|||e|eu||+||ou, .+||eº |u ||º +çç|eºº|.e·
ueºº.
I|e |eº|ou |º + p|+que o| + uodu|e W||| .+|,|uç
deç|eeº o| |e|+||u|/+||ou |u ||e uodu|e +ud/o| ou
||e ºu||+ce. I|ur| |u|e. uud|||e|eu||+|ed 'CC |º
ºo|| +ud |+º uo |,pe||e|+|oº|º, d|||e|eu||+|ed 'CC
|º |+|d ou p+|p+||ou +ud |+º |,pe||e|+|oº|º.
I|e r+jo|||, o| u\k·|uduced |eº|ouº |+.e + |oW
|+|e o| d|º|+u| re|+º|+º|º |u o||e|W|ºe |e+|||,
|ud|.|du+|º. \o|e +çç|eºº|.e 'CC º occu| |u |rru·
uoºupp|eººed |ud|.|du+|º W||| + ç|e+|e| |uc|deuce
o| re|+º|+º|º.
I|e+|reu| |º |, ºu|çe|,.
|C|·9 . ¹1!.0
°
|C|·¹0 . \301o/2·!
INvASIv£ SÇüAM0üS C£II CAkCIN0MA (SCC)
£FI0£MI0I0C¥ AN0 £II0I0C¥
ü|travìo|et kadìatìoo
A¿e oI 0oset Oldei than 55 yeais of age in
the United States; in Austialia, New Zealand,
in Floiida, Southwest and Southein Califoinia
peisons in theii twenties and thiities.
Iocìdeoce Continental United States: 12 pei
100,000 white males; 7 pei 100,000 white fe-
males. Hawaii: 62 pei 100,000 whites.
Sex Males > females, but SCC can occui moie
fiequently on the legs of females.
£xposure Sunlight. Phototheiapy, PUVA (oial
psoialen - UVA). Excessive photochemotheiapy
can lead to piomotion of SCC, paiticulaily in pa-
tients with skin phototypes I and II oi in patients
with histoiy of pievious exposuie to ionizing ia-
diation oi methotiexate tieatment foi psoiiasis.
kace Peisons with white skin and pooi tan-
ning capacity (skin phototypes I and II) (see
Section 10). Biown- oi black-skinned peisons
can develop SCC fiom numeious etiologic
agents othei than UVR.
Ceo¿raphy Most common in aieas that have
many days of sunshine annually, i.e., in Aus-
tialia and southwestein United States.
0ccupatìoo Peisons woiking outdoois-faim-
eis, sailois, lifeguaids, telephone line installeis,
constiuction woikeis, dock woikeis.
humao Fapì||omavìrus
Oncogenic HPV type-16, -18, -31 most com-
monly, -33, -35, -39, -40, and -51 to -60 aie
associated with epithelial dysplasia, SCCIS, and
invasive SCC. HPV-5, -8, -9 have also been iso-
lated fiom SCCs.
0ther £tìo|o¿ìc Factors
Immuoosuppressìoo Solid oigan tiansplant
iecipients, individuals with chionic immuno-
suppiession of inflammatoiy disoideis, and
those with HIV disease aie associated with an
incieased incidence of UVR- and HPV-induced
SCCIS and invasive SCCs. SCCs in these indi-
viduals aie moie aggiessive than in nonimmu-
nosuppiessed individuals.
Chrooìc IoI|ammatìoo Chionic cutaneous lu-
pus eiythematosus, chionic ulceis, buin scais,
chionic iadiation deimatitis, lichen planus of
oial mucosa.
Iodustrìa| Carcìoo¿eos Pitch, tai, ciude paiaf-
fin oil, fuel oil, cieosote, lubiicating oil, nitio-
souieas.
Ioor¿aoìc Arseoìc Tiivalent aisenic had been
used in the past in medications such as Asiatic
pills, Donovan pills, Fowlei solution (used as a
tieatment foi psoiiasis). Histoiically tiivalent ai-
senic was used foi tieatment of psoiiasis. Aisenic
is still piesent in diinking watei in some geo-
giaphic iegions (West Bengal and Bangladesh).
CIINICAI MANIF£SIAII0N
Slowly evolving-any isolated keiatotic oi
eioded papule oi plaque in a suspect patient
that peisists foi ovei a month is consideied a
caicinoma until pioved otheiwise. Also, a nod-
ule evolving in a plaque that meets the clinical
ciiteiia of SCCIS (Bowen disease), a chionically
eioded lesion on the lowei lip oi on the penis,
oi nodulai lesions evolving in oi at the maigin
of a chionic venous ulcei oi within chionic
iadiation deimatitis. Note that SCC is always
asymptomatic. Potential caicinogens often can
be detected only aftei detailed inteiiogation of
the patient.
Rapidly evolving-invasive SCC can eiupt
within a few weeks and is often painful and/oi
tendei.
Foi didactic ieasons, two types can be distin-
guished:
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 281
1. Highly diffeientiated SCCs, which piactically
always show signs of keiatinization eithei
within oi on the suiface (hypeikeiatosis)
of the tumoi. These aie fiim oi haid upon
palpation (Figs. 11-6 to 11-8 and Figs. 11-10
to 11-12).
2. Pooily diffeientiated SCCs, which do not
show signs of keiatinization and clinically
appeai fleshy, gianulomatous, amd conse-
quently aie soft upon palpation (Figs. 11-5
and 11-9).
FICük£ 11-T Squamous ce|| carcìooma A |ouud uodu|e, |||r +ud |udo|eu| W||| + ceu||+| ||+c| eºc|+|.
|o|e ,e||oW|º| co|o| |u ||e pe||p|e|, o| ||e |uro| |ud|c+||uç ||e p|eºeuce o| |e|+||u. A|| |||ee 'CC º|oWu |u ||ç.
¹¹·o +ud |e|e +|e |+|d +ud occu| ou ||e |oWe| ||p. 'CC |+|d|, occu|º ou ||e uppe| ||p |ec+uºe |||º |º º|+ded
||or ||e ºuu. 'CC ou ||e ||p |º e+º||, d|º||uçu|º|ed ||or uodu|+| BCC |ec+uºe BCC doeº uo| de.e|op |,pe||e|+·
|oº|º o| |e|+|oº|º |uº|de ||e |uro| +ud doeº uo| occu| ou ||e .e|r|||ou ||p.
FICük£ 11-6 Squamous ce|| carcìooma: ìovasìve oo the |ìp, two sta¿es oI deve|opmeot  A |+|çe
|u| ºu|||e uodu|e, W||c| |º |e||e| |e|| ||+u ºeeu, ou ||e .e|r|||ou |o|de| o| ||e |oWe| ||p W||| +|e+º o| |,pe||e|+·
|oº|º +ud e|oº|ou, +||º|uç |u ||e ºe|||uç o| de|r+|o|e||oº|º o| ||e ||p (c|e|||||º +c||u|c+).  I||º uodu|e |º |+|çe| +ud
c+u |e |e|| |o |u|||||+|e ||e eu|||e ||p.
 
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 282
0ìIIereotìated SCC
Iesìoos Induiated papule, plaque, oi nod-
ule (Figs. 11-1, 11-6 to 11-8); adheient thick
keiatotic scale oi hypeikeiatosis (Figs. 11-1,
11-6, 11-7, 11-8, 11-11); when eioded oi ulcei-
ated, the lesion may have a ciust in the centei
and a fiim, hypeikeiatotic, elevated maigin
(Figs. 11-7 and 11-8). Hoiny mateiial may be
expiessed fiom the maigin oi the centei of the
lesion (Figs. 11-7, 11-8, and 11-10). Eiythema-
tous, yellowish, skin coloi. Haid. Polygonal,
oval, iound (Figs. 11-6 and 11-10), oi umbili-
cated and ulceiated.
DIstrIhutIvn Usually isolated but may be
multiple. Usually exposed aieas. Sun-induced
keiatotic and/oi ulceiated lesions especially on
the bald scalp (Fig. 11-1), cheeks, nose, lowei
lips (Fig. 11-6), eais (Fig. 11-11), pieauiiculai
aiea, doisa of the hands (Fig. 11-10), foieaims,
tiunk, and shins (females) (Fig. 11-12).
0ther Fhysìca| Fìodìo¿s Regional lymphaden-
opathy due to metastases.
Specìa| Features In UV-ielated SCC evidence
of Jermaìo|e|íosís and so|ar |eraìoses . SCCs of
the lips develop fiom leukoplasia oi actinic
cheilitis; in 90% of cases they aie found on
the lowei lip (Fig. 11-6). In chionic iadio-
deimatitis they aiise fiom iadiation-induced
keiatoses (see Fig. 10-34); in individuals with
a histoiy of chionic intake of aisenic, fiom
aisenical keiatoses. Diffeientiated (i.e., hypei-
keiatotic) SCC due to HPV on genitalia; SCC
due to excessive PUVA theiapy on lowei ex-
tiemities (pietibial) oi on genitalia. SCCs in
scais fiom buins, in chionic stasis ulceis of
long duiation, and in sites of chionic inflam-
mation aie often difficult to identify. Suspicion
is indicated when nodulai lesions aie haid and
show signs of keiatinization (Figs. 11-8, 11-10,
and 11-11).
S¡etía| [orm : caicinoma cuniculatum, usually
on the soles, highly diffeientiated, HPV-ielated
but can also occui in othei settings (Fig. 11-13).

hìstopatho|o¿y SCCs with vaiious giades of
anaplasia and keiatinization.
üodìIIereotìated SCC
Iesìoos Fleshy, gianulating, easily vulneiable,
eiosive papules and nodules and papilloma-
tous vegetations (Fig. 11-9). Ulceiation with
a neciotic base and soft, fleshy maigin. Bleeds
easily, ciusting. Red. Soft. Polygonal, iiiegulai,
often cauliflowei-like.
DIstrIhutIvn Isolated but also multiple, pai-
ticulaily on the genitalia, wheie they aiise fiom
eiythioplasia (see Fig. 35-24) and on the tiunk
Claik level I, intiaepideimal; level II, invades papil-
laiy deimis; level III fills papillaiy deimis; level IV,
invades ieticulai deimis; level V
,
invades subcutane-
ous fat.
(Fig. 11-5), lowei extiemities, oi face, wheie
they aiise fiom Bowen disease.
Mìsce||aoeous 0ther Skìo Chao¿es Lymphad-
enopathy as evidence of iegional metastases
is fai moie common than with diffeientiated,
hypeikeiatotic SCCs.
hìstopatho|o¿y Anaplastic SCC with multiple
mitoses and little evidence of diffeientiation
and keiatinization.
0IFF£k£NIIAI 0IACN0SIS
As stated pieviously, any peisistent nodule,
plaque, oi ulcei, but especially when these occui
in sun-damaged skin, on the lowei lips, in aieas
of iadiodeimatitis, in old buin scais, oi on the
genitalia, must be examined foi SCC. Keiato-
acanthoma may be clinically indistinguishable
fiom diffeientiated SCC (Fig. 11-8 À ).
MANAC£M£NI
Sur¿ery Depending on localization and extent
of lesion, excision with piimaiy closuie, skin
flaps, oi giafting. Micioscopically contiolled
suigeiy in difficult sites. Radiotheiapy should
be peifoimed only if suigeiy is not feasible.
C0ükS£ AN0 Fk0CN0SIS
kecurreoce aod Metastases SCC causes local
tissue destiuction but it has a significant poten-
tial foi metastases. Metastases aie diiected to ie-
gional lymph nodes and appeai 1 to 3 yeais aftei
initial diagnosis. In-tiansit metastases occui. In
solid oigan tiansplant iecipients, can be piesent
when SCC is diagnosed/detected oi shoitly aftei.
SCC in the skin has an oveiall metastatic iate of
3-4% and tends to occui with tumois that aie
laige, iecuiient, and involve deep stiuctuies of
cutaneous neives. High-iisk SCCs aie defined as
having a diametei >2 cm, a level of invasion >
4 mm, and Claik levels IV oi V ; tumoi involve-
ment of bone, muscle, and neive (so-called
neuiotiopic SCC, occuis fiequently on the
foiehead and scalp); location on eai, lip, and
genitalia; tumois aiising in a scai oi following
ionizing iadiation aie usually highly dediffeien-
tiated tumois. Canceis aiising in chionic osteo-
myelitis sinus tiacts, in buin scais, and in sites
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 283
of iadiation deimatitis have a metastatic iate
of 31, 20, and 18%, iespectively. On the othei
hand, SCC aiising in solai keiatoses have the
lowest potential foi metastasis. A special gioup
of high-iisk SCCs aie those in patients who aie
immunosuppiessed (Fig. 11-14).
SCCs ìo Immuoosuppressìoo Oigan tians-
plant iecipients have a maikedly incieased
incidence of NMSCs, piimaiily SCC, which is
40 to 50 times gieatei than in the geneial popu-
lation. Risk factois include skin type, cumula-
tive sun exposuie, age at tiansplantation, male
sex, HPV infections, the degiee and length of
immunosuppiession, and the type of immuno-
suppiessant. Lesions aie often multiple, usually
in sun-exposed sites but also in the genital,
anal, and peiigenital iegions (Fig. 11-14).
These tumois giow iapidly and aie aggies-
sive; in one seiies of heait-tiansplant patients
fiom Austialia, 27% died of skin cancei.
Patients with AIDS have only a slight in-
cieased iisk of NMSC. In one seiies a fouifold
inciease in theii iisk of developing lip SCC
was noted. Howevei, SCC of the anus is sig-
nificantly incieased in this population (see also
Section 21).
FICük£ 11-8 Squamous ce|| carcìooma, we|| dìIIereotìated  A uodu|e ou ||e |oWe| +|r co.e|ed
W||| + dore·º|+ped d+|| |,pe||e|+|oº|º.  A |+|çe, |ouud, |+|d uodu|e ou ||e uoºe W||| ceu||+| |,pe||e|+|oº|º.
|e|||e| |eº|ou c+u |e d|º||uçu|º|ed ||or |e|+|o+c+u||or+ (ºee ||ç. ¹¹·¹5).
 
FICük£ 11-9 Squamous ce||
carcìooma, uodìIIereotìated I|e|e |º
+ c||cu|+|, dore·º|+ped |edd|º| uodu|e
W||| p+|||, e|oded ºu||+ce ou ||e |erp|e
o| + 13·,e+|·o|d r+|e. I|e |eº|ou º|oWº
uo |,pe||e|+|oºeº +ud |º ºo|| +ud |||+||e.
w|eu ºc|+ped || ||eedº e+º||,.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 284
FICük£ 11-10 Squamous ce|| carcìooma, advaoced, we|| dìIIereotìated, oo the haod oI a 65-year-
o|d Iarmer I|e ||ç uodu|e |º ºroo||, .e|, |+|d upou p+|p+||ou, +ud º|oWº + ,e||oW|º| co|o|, |oc+||, |ud|c+||uç
|e|+||u |u ||e |od, o| ||e uodu|e. || ||e |eº|ou We|e |uc|ºed |u ||e ,e||oW|º| +|e+º, + ,e||oW|º|·W|||e r+|e||+|
(|e|+||u) cou|d |e e\p|eººed.
FICük£ 11-11 Squamous ce|| carcìooma, hì¿h|y dìIIereotìated, oo the ear I|e|e |º + |e|+||.e|, |+|çe
p|+que co.e|ed |, +d|e|eu| |+|d |,pe||e|+|oºeº. A|||ouç| 'CCº +|e |u çeue|+| uo| p+|u|u|, |eº|ouº ou ||e |e||\ o|
+u||e||\ uºu+||, +|e, +º W+º ||e c+ºe |u |||º o9·,e+|·o|d r+u.
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 285
FICük£ 11-13 Squamous ce|| carcìooma (carcì-
ooma cuoìcu|atum) ìo a patìeot wìth perìphera|
oeuropathy due to |eprosy A |+|çe |uuç+||uç,
p+|||+||, uec|o||c +ud |,pe||e|+|o||c |uro| ou ||e ºo|e o|
||e |oo|. I|e |eº|ou |+d |eeu couº|de|ed + ueu|op+|||c
u|ce|, +ºc|||ed |o |ep|oº,, |u| cou||uued ç|oW|uç +ud
|ec+re e|e.+|ed +ud u|ce|+|ed.
FICük£ 11-12 Squamous ce|| carcìooma ìo
the settìo¿ oI chrooìc statìs dermatìtìs aod
|oo¿-staodìo¿, ooohea|ìo¿ veoous u|cer I|e|e
W+º + .euouº u|ce| o| ro|e ||+u ¹0 ,e+|º' du|+||ou
+| ||e º||e, W||c| W+º uuºucceºº|u||, ||e+|ed W|||
|op|c+| |ered|eº. C|+du+||, ||e ceu|e| o| ||e u|ce|
|ec+re |+|de| +ud e|e.+|ed +ud uoW |ep|eºeu|º +
|||r e|e.+|ed, e+º||, ||eed|uç r+ºº W||| ,e||oW uec|o·
ºeº. |ouç·º|+ud|uç u|ce|º o| ||e |eç º|ou|d +|W+,º |e
||opº|ed |o |u|e ou| ºqu+rouº ce|| c+|c|uor+.
FICük£ 11-14 Squamous ce|| carcìooma ìo a
reoa| traosp|aot recìpìeot oo the base oI the
scrotum |u +dd|||ou |o |||º u|ce|+||uç |||r uodu|e
o| ||e |+ºe o| ||e ºc|o|ur, ||e p+||eu| |+d ºr+||e|,
º|r||+| |eº|ouº e|ºeW|e|e ou ||e |od,. '|uce |e |+d
pºo||+º|º +ud |+d ||e|e|o|e ºpeu| couº|de|+||e ||re
|u ||e ºuu, ||e |eº|ouº |u ||e ºuu·e\poºed º|deº We|e
p|o|+||, due |o u\k. I|e |eº|ou º|oWu |e|e W+º
p|o|+||, |u|||+|ed |, n|\ +º |e |+d + º|r||+| |eº|ou
pe||+u+||, +ud ou ||e ç|+uº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 286
£FI0£MI0I0C¥
A¿e oI 0oset Ovei 50 yeais; iaie below 20
yeais. Male:female iatio 2:1.
FAIh0C£N£SIS
Human papillomaviius (HPV) -9, -16, -19, -25,
and -37 have been identified in KAs. Othei
possible etiologic factois include UV iadiation
and chemical caicinogens (industiial: pitch
and tai).
CIINICAI MANIF£SIAII0N
Rapid giowth, achieving a size of 2.5 cm within
a few weeks. No symptoms, but theie aie occa-
sional tendeiness and cosmetic disfiguiement.
Skìo Iesìoos Nodule, dome-shaped, often
with a cential keiatotic plug (Fig. 11-15). Skin-
coloied oi slightly ied, tan/biown. Fiim but not
haid. 2.5 cm (iange, 1-10 cm), iound. Keiatotic
plug may appeai like a cutaneous hoin. Re-
moval of plug iesults in a ciatei.
DIstrIhutIvn Isolated single lesion. Uncom-
monly, may be multiple, eiuptive. On exposed
skin: cheeks, nose, eais, hands (doisa).
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y A iepiesentative biopsy that
extends thiough the entiie lesion to pieseive the
KA |º + ºpec|+| |eº|ou, |o|re||, couº|de|ed + pºeu·
doc+uce| || |º uoW |eç+|ded |, roº| +º + .+||+u|
o| ºqu+rouº ce|| c+|c|uor+.
A |e|+||.e|, corrou, |+p|d|, ç|oW|uç ep|||e||+|
|uro| W||| po|eu||+| |o| ||ººue deº||uc||ou +ud
(|+|e) re|+º|+º|º, |oWe.e|, |u roº| c+ºeº ºpou|+·
ueouº |eç|eºº|ou.
A dore·º|+ped uodu|e W||| ceu||+| |e|+|o||c p|uç
(||ç. ¹¹·¹5 1 ).
||ed||ec||ou |o| ºuu·e\poºed º||eº.
\u|||p|e KAº occu|.
I|e+|reu| |º |, e\c|º|ou.
k£kAI0ACANIh0MA (kA) |C|·9 . 2!3.2
°
|C|·¹0 . |53.3
aichitectuie of the nodule oi piimaiy excision
is iequiied. Cential, laige, iiiegulaily shaped
ciatei filled with keiatin. The suiiounding
epideimis extends in a liplike mannei ovei the
sides of the ciatei. The keiatinocytes aie atypi-
cal and many aie dyskeiatotic. Diffeientiation
of KA fiom highly diffeientiated SCC is difficult
and may not always be possible.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical findings confiimed by iepiesentative
biopsy. SCC, hypeitiophic solai keiatosis, vei-
iuca vulgaiis.
C0ükS£ AN0 Fk0CN0SIS
Spontaneous iegiession in 2-6 months oi
sometimes >1 yeai in most cases. Theie is pio-
giessive keiatinization with expansion of the
cential keiatotic plug until all epithelial tumoi
tissue is conveited into hoiny mateiial and shed
(Figs. 11-15 B and 11-15 C ) which leads to a scai.
Howevei, KA is locally destiuctive; lymph node
and visceial metastases have been obseived in
some cases.
MANAC£M£NI
Sur¿ery Suigical excision is iecommended in
that KA cannot be distinguished fiom SCC on
clinical findings.
Mu|tìp|e kAs Systemic ietinoids and meth-
otiexate have been used.
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 28T
£FI0£MI0I0C¥
A¿e oI 0oset Oldei than 40 yeais.
Sex Males > females.
Iocìdeoce United States: 500-1000 pei
100,000, highei in the sunbelt; >400,000 new
patients annually.
kace Raie in biown- and black-skinned pei-
sons.
£II0I0C¥
UVR, mostly of the UVB spectium (290-320
nm) that induces mutations in suppiessoi
genes. The piopensity foi multiple BCC may
be inheiited. Associated with mutations in the
PTCH gene in many cases.
Fredìsposìo¿ Factors Skin phototypes I and
II and albinos aie highly susceptible to develop
BCC with piolonged sun exposuie. Also a his-
toiy of heavy sun exposuie in youth piedisposes
the skin to the development of BCC latei in
BCC |º ||e roº| corrou c+uce| |u |ur+uº.
C+uºed |, u\k, |íC| çeue ru|+||ou |u roº|
c+ºeº.
C||u|c+||, d|||e|eu| |,peº. uodu|+|, u|ce|+||uç, p|ç·
reu|ed, ºc|e|oº|uç, +ud ºupe|||c|+|.
BCC |º |oc+||, |u.+º|.e, +çç|eºº|.e, +ud deº||uc||.e
|u| º|oW ç|oW|uç, +ud ||e|e |º .e|, ||r||ed (|||e|·
+||, uo) |eudeuc, |o re|+º|+º|/e.
I|e+|reu| |º |, ºu|ç|c+| e\c|º|ou, \o|º r|c|o·
ç|+p||c ºu|çe|,, e|ec||odeº|cc+||ou, +ud cu|e||+çe.
A|ºo c|,oºu|çe|, +ud |r|qu|rod c|e+r.
8ASAI C£II CAkCIN0MA (8CC) |C|·9 . ¹1!.0
°
|C|·¹0 . C!!.\3090/!
life. Pievious theiapy with x-iays foi facial acne
gieatly incieases the iisk of BCC, even in those
peisons with a good ability to tan (skin photo-
types III and IV). Supeificial multicentiic BCC
occuis 30-40 yeais aftei ingestion of aisenic but
also without appaient cause.
CIINICAI MANIF£SIAII0N
Slowly evolving, usually asymptomatic. Eiosion
oi bleeding with minimal tiauma may be fiist
symptom.
Skìo Iesìoos Theie aie five t|íníta| types:
nodulai, ulceiating, scleiosing (cicatiicial),
supeificial, and pigmented.
· NoJu|ar BCC. Papule oi nodule, tianslucent
oi °peaily." Skin-coloied oi ieddish, smooth
suiface with telangiectasia, well defined, fiim
(Figs. 11-16 and 11-17). Poitions of nodulai
BCC may have eiosions oi stipples of melanin
pigmentation.
 
FICük£ 11-15 keratoacaothoma showìo¿ dìIIereot sta¿es oI evo|utìoo  |u|||+||, ||e|e |º + |ouud
dore·º|+ped, .e|, |||r uodu|e, |edd|º| W||| + ceu||+| |,pe||e|+|o||c p|uç. I||º |+º |eeu p+|||+||, º|ed |e+.|uç +
ceu||+| c|+|e|  n,pe||e|+|oº|º |+º p|oç|eººed +ud |+º uoW |ep|+ced roº| o| ||e uodu|e, |e+.|uç ou|, + |||u ||r
o| |uro| ||ººue |u ||e pe||p|e|,.  |u|||e| p|oç|eºº|ou o| |,pe||e|+|oºeº +ud |e|+||u|/+||ou |+º uoW |ep|+ced +||
o| ||e |uro| +ud W||| |e |+|e| º|ed, |e+.|uç + ºc+|. '|uce |||º e.o|u||ou |º uo| +|W+,º p|ed|c|+||e, |e|+|o+c+u||or+
º|ou|d +|W+,º |e e\c|ºed |u ||e e+||, º|+çeº.

FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 288
· U|teraìíng BCC. Ulcei (often coveied with a
ciust) with a iolled boidei (iodent ulcei), which
again is tianslucent, peaily, smooth with tel-
angiectasia, and fiim (Figs. 11-18 and 11-19).
· St|erosíng BCC. Appeais as a small patch
of moiphea oi a supeificial scai, often ill-
defined, skin-coloied, whitish but also with
peppeiy pigmentation (Fig. 11-20). In this
infiltiating type of BCC theie is an excessive
amount of fibious stioma. Histologically,
fingei-like stiands of tumoi extend fai into
the suiiounding tissue, and excision theie-
foie iequiies wide maigins. Scleiosing BCC
can piogiess to nodulai oi ulceiating BCC
(Figs. 11-20 B and 11-21).
· Su¡er[ítía| mu|ìítenìrít BCCs. Appeai as thin
plaques (Figs. 11-22 and 11-23). Pink oi
ied; chaiacteiistic fine thieadlike boidei and
telangiectasia can be seen with the aid of a
hand lens. This is the only foim of BCC that
can exhibit a consideiable amount of scaling.
This can also give iise to nodulai and ulcei-
ating BCC (Fig. 11-23). BCC often bleeds
with minimal excoiiation by fingeinail. Solai
keiatosis, in compaiison, does not bleed but is
somewhat painful with excoiiation.
· PígmenìeJ BCC. May be biown to blue oi
black (Fig. 11-24). Smooth, glistening suiface;
haid, fiim; may be indistinguishable fiom
supeificial spieading oi nodulai melanoma
but is usually haidei. Cysìít lesions may oc-
cui: iound, oval shape, depiessed centei
(°umbilicated"). Stippled pigmentation can
be seen in any of BCC types.
DIstrIhutIvn (Image 11-2) Isolated single le-
sion; multiple lesions aie not infiequent; > 90%
occui in the face. Seaich caiefully foi °dangei
sites": medial and lateial canthi (Fig. 11-17 À ,
B , C ), nasolabial fold (Fig. 11-16 B ), behind
the eais (Figs. 11-18 B and 11-19). Supeificial
multicentiic BCCs occui on the tiunk (Figs.
11-22 and 11-23). BCC usually aiises only fiom
epideimis that has a capacity to develop (haii)
follicles. Theiefoie, BCCs iaiely occui on the
veimilion boidei of the lips oi on the genital
mucous membianes.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Solid tumoi consisting of
piolifeiating atypical basal cells, laige, oval,
deep-blue staining on H&E, but with little
anaplasia and infiequent mitoses; palisading
aiiangement at peiipheiy; vaiiable amounts of
mucinous stioma.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Seiious BCCs occuiiing in the dangei sites
(cential pait of the face, behind the eais) aie
ieadily detectable by caieful examination with
good lighting, a hand lens, and caieful palpa-
tion and deimoscopy. Diagnosis is made clini-
cally and confiimed micioscopically. Diffeiential
FICük£ 11-16 8asa| ce|| carcìooma: oodu|ar type  A ºr+|| pe+||, p+pu|e (+||oW) ou ||e uoº|||| +ud
+u e.eu ºr+||e| oue (ºr+|| +||oW) |u ||e u+ºo|+||+| |o|d. I|eºe +|e .e|, e+||, º|+çeº o| BCC. I|e ç|+, +||oW
deuo|eº + de|r+| |\|.  I||º |º + |u|||e| +d.+uced uodu|+| BCC. A ºo|||+|,, º||u,, uodu|e W||| |+|çe |e|+uç|ec·
|+||c .eººe|º ou ||e +|+ u+º|, +||º|uç ou º||u W||| de|r+|o|e||oº|º.


S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 289
diagnosis includes all smooth papules such as
deimal nevomelanocytic nevi, tiichoepithelioma,
deimatofibioma, and otheis; if pigmented, su-
peificial spieading and nodulai melanoma; if ul-
ceiated, all nonpainful fiim ulceis including SCC
and a (extiagenital) piimaiy chancie of syphilis.
MANAC£M£NI
Excision with piimaiy closuie, skin flaps, oi
giafts. Ciyosuigeiy and electiosuigeiy aie op-
tions, but only foi veiy small lesions and not in
the dangei sites oi on the scalp.
FICük£ 11-1T 8asa| ce|| carcìooma: oodu|ar type  A ç||º|eu|uç, ºroo|| p|+que ou ||e |oWe| e,e||d
W||| ru|||p|e |e|+uç|ec|+º|+º.  Au o.+|, pe+||, uodu|e ou ||e uoºe c|oºe |o ||e |uue| c+u||uº.  A ºroo||,
pe+||, |uro| W||| |e|+uç|ec|+º|+ |e|oW ||e |oWe| e,e||d. Iuro| |ee|º |+|d, |º We|| de||ued, +ud |º +º,rp|or+||c.
 A |+|çe, |||r |edd|º| ç||º|eu|uç uodu|e W||| ºr+|| u|ce|+||ouº ou ||e uoºe.
C 0
 
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 290
Foi lesions in the dangei sites (nasolabial
aiea, aiound the eyes, in the eai canal, in the
posteiioi auiiculai sulcus, and on the scalp)
and scleiosing BCC, micioscopically contiolled
suigeiy (Mohs suigeiy) is the best appioach.
Radiation theiapy is an alteinative only when
disfiguiement may be a pioblem with suigical
excision (e.g., eyelids oi laige lesions in the na-
solabial aiea) oi in veiy old age.
Theie aie a vaiiety of topical tieatments that
can be used foi supeificial BCCs but only foi
those tumois below the neck; tryosurgery is ef-
fective but leaves a white scai that iemains foi
life. Electiocauteiy with cuiettage is also simple
and effective, but it leaves scais and should
be used only in small lesions. Topical 5-fluoi-
ouiacil ointment and imiquimod cieam foi
supeificial BCC, 5 times a week, foi 6 weeks, aie
effective, do not cause scais, but iequiie con-
sideiable time and may not iadically iemove all
tumoi tissue. Imiquimod iequiies compliance
by patient oi caiegivei. Imiquimod is especially
FICük£ 11-18 8asa| ce|| carcìooma, u|cerated: kodeot u|cer  A |+|çe c||cu|+| u|ce| ou ||e ||p o|
||e uoºe W||| + W+||·|||e |o|de|.  A º|r||+| |eº|ou |u ||e |e||o+u||cu|+| |eç|ou. I|e|e |º + |o||ed pe+||, |o|de|
ºu||ouud|uç ||e u|ce|.  kodeu| u|ce| |u ||e p|e+u||cu|+| |eç|ou. A |o||ed pe+||, |o|de| ºu||ouudº +u u|ce| W|||
,e||oW uec|oºeº +ud + ||u, ||+c| c|uº|.  A deep u|ce| W||| + ºu||ouud|uç |o||ed |o|de|, ºroo||, ç||º|eu|uç +ud
p+|||, co.e|ed W||| c|uº|º |u ||e r+ud||u|+| |eç|ou. A|| ||eºe |eº|ouº +|e |+|d upou p+|p+||ou.
C 0
 
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 291
FICük£ 11-19 A |ar¿e rodeot u|cer ìo the oucha| aod retroaurìcu|ar area exteodìo¿ to the temp|e
I|e eu|||e |eº|ou couº|º|º o| + |||r ç|+uu|+||uç ||ººue, p+|||+||, co.e|ed |, |ero|||+ç|c c|uº|º. I|e d|+çuoº|º c+u
|e r+de ou|, |, e\+r|u|uç ||e |o|de|, W||c| |º |o||ed, e|e.+|ed, |||r, +ud ºroo||.
FICük£ 11-20 8asa| ce|| carcìooma: sc|erosìo¿ type  A ºr+|| |ucouºp|cuouº +|e+ |eºer|||uç ºupe|||c|+|
ro|p|e+, ||| de||ued, ,e||oW|º| W||| |e|+uç|ec|+º|+. upou p+|p+||ou, |oWe.e|, + p|+|e|||e |udu|+||ou c+u |e |e|| +ud
|||º e\|eudº |e,oud ||e .|º|||e r+|ç|uº o| ||e |eº|ou. A||e| .e||||c+||ou o| ||e d|+çuoº|º |, ||opº,, || W||| |equ||e
e\c|º|ou W||| W|de r+|ç|uº.  A |+|çe dep|eººed +|e+ |eºer|||uç + ºc+| ou ||e uoºe, ou ||e ||ç|| (|+|e|+|) +ud
red|+| r+|ç|uº o| |||º 'ºc+|¨ ||e|e |º ||e |,p|c+| |o||ed |o|de| o| + uodu|+| BCC. I||º |eº|ou |º º|oWu |o derou·
º||+|e ||+| ºc|e|oº|uç +ud uodu|+| BCC +|e º|rp|, |Wo d|||e|eu| ç|oW|| p+||e|uº.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 292
FICük£ 11-21 8asa| ce||
carcìooma, sc|erosìo¿, oodu|ar,
aod u|ceratìo¿ A |+|çe |eº|ou,
W||c| |oo|º |||e ro|p|e+ +ud |º
W||||º| +ud |||r upou p+|p+||ou
|u| W||||u ||e |e.e| o| ||e º||u, |º
|ouud ou ||e |erp|e +ud |u ||e
ºup|+c|||+| |eç|ou. w||||u |eº|ou
+ud +| ||e r+|ç|uº ||e|e +|e ºr+||
uodu|eº o| BCCº. 0u ||e |+|e|+|
c+u||uº o| ||e e,e ||e|e |º + |+|çe
u|ce| W||| |o||ed |o|de|º |ep|eºeu|·
|uç |odeu| u|ce|. Aç+|u |||º ||çu|e |º
º|oWu |o derouº||+|e ||+| ||e d||·
|e|eu| |,peº o| |+º+| ce|| c+|c|uor+
+|e juº| d|||e|eu| ç|oW|| p+||e|uº.
FICük£ 11-22 SuperIìcìa| basa| ce|| carcìooma: so|ìtary |esìoo aod mu|tìp|e |esìoos  I||º |||ç||
|ed |eº|ou |+º + º||ç|||, e|e.+|ed |o||ed |o|de| ||+| c+u |e de|ec|ed W||| 'º|de ||ç|||uç¨, +|||ouç| |||º |eº|ou |º
|,p|c+| euouç| |o |e d|+çuoºed c||u|c+||,, + ||opº, |º ueceºº+|, |o .e|||, ||e d|+çuoº|º.  \+u, ºupe|||c|+| |+º+|
ce|| c+|c|uor+º ou ||e ||uu|. I|e, +ppe+| +º |||ç|||, e|,||er+|ouº, o||eu ºc+||uç, ||+| |eº|ouº, o||eu W|||ou| +
|o||ed |o|de|. I|e |,pop|çreu|ed +|e+º |ep|eºeu| ºupe|||c|+| ºc+|º +||e| c|,o||e|+p, o| ºupe|||c|+| BCCº.
â 8
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 293
good foi young peisons who do not want scais.
Photodynamic theiapy is effective only in veiy
supeificial lesions and iadiation sessions (pho-
todynamic dye - visible light) aie painful.
C0ükS£ AN0 Fk0CN0SIS
BCC does not metastasize. The ieason foi
this is the tumoi's giowth dependency on its
stioma, which on invasion of tumoi cells into
the vessels is not disseminated with the tumoi
cells. When tumoi cells lodge at distant sites,
they do not multiply and giow because of the
absence of giowth factois deiived fiom theii
stioma. Exceptions occui when a BCC shows
signs of dediffeientiation, foi instance, aftei
inadequate iadiotheiapy. Most lesions aie iead-
ily contiolled by vaiious suigical techniques.
Seiious pioblems, howevei, may occui with
BCC aiising in the dangei sites of the head. In
these sites the tumoi may invade deeply, cause
extensive destiuction of muscle and bone, and
even invade to the duia matei. In such cases,
death may iesult fiom hemoiihage of eioded
laige vessels oi infection.
FICük£ 11-23 SuperIìcìa| basa| ce|| carcìooma, ìovasìve I|e|e +|e |Wo |||eçu|+| |ed +|e+º W||| |o||ed
|o|de|º +ud ceu||+| |e|+uç|ec|+º|+. |u ||e |+|çe| |eº|ou ||e BCC |º e|e.+|ed W||| +u |||eçu|+| ºu||+ce +ud uoW
+ººureº ||e ro|p|o|oç, +ud ç|oW|| |e|+.|o| o| + uodu|+| BCC, ou ||e ||ç|| ||e |eº|ou |º e|oº|.e +ud W|||
p|oç|eºº |o +u u|ce|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 294
I||º +u|oºor+| dor|u+u| d|ºo|de| |º c+uºed |,
ru|+||ouº |u ||e p+|c|ed çeue ||+| |eº|deº ou
c||oroºore 9q (9q22).
|| +||ec|º º||u W||| ru|||p|e BCCº +ud ºo·c+||ed
p+|rop|+u|+| p||º +ud |+º + .+||+||e e\p|eºº|ou o|
+|uo|r+||||eº |u + uur|e| o| º,º|erº, |uc|ud|uç
º|e|e|+| r+||o|r+||ouº, ºo|| ||ººue, e,eº, C|', +ud
eudoc||ue o|ç+uº.
I|e º,ud|ore occu|º roº||, |u W|||eº |u| +|ºo |u
||oWu· +ud ||+c|·º||uued peop|e, +ud ||e|e |º +u
equ+| ºe\ |uc|deuce.
BCCº |eç|u º|uç|, |u c|||d|ood o| e+||, +do|eº·
ceuce +ud cou||uue |||ouç|ou| |||e.
I|e|e +|e ro|e BCCº ou ||e ºuu·e\poºed +|e+º o|
||e º||u, |u| ||e, +|ºo occu| |u co.e|ed +|e+º +ud
||e|e r+, |e |uud|edº o| |eº|ouº.
C|+|+c|e||º||c çeue|+| |e+|u|eº +|e ||ou|+| |oºº·
|uç, + ||o+d u+º+| |oo|, +ud |,pe||e|o||ºr. A
º,º|erº |e.|eW r+, |e.e+| couçeu||+| +uor+||eº
|uc|ud|uç uudeºceuded |eº|eº +ud |,d|ocep|+|uº.
0||e| -·|·c:±|c¤-¤±º |-º·¤¤º +|e r+ud||u|+| j+W
odou|oçeu|c |e|+|oc,º|º, W||c| r+, |e ru|||p|e
+ud r+, |e uu||+|e|+| o| |||+|e|+|. I|e|e r+, |e
de|ec||.e deu||||ou, ||||d o| ºp|+,ed |||º, pec|uº
e\c+.+|ur, º|o|| |ou||| re|+c+|p+|º, ºco||oº|º,
+ud |,p|oº|º. E,e |eº|ouº |uc|ude º||+||ºruº,
|,pe||e|o||ºr, d,º|op|+ c+u||o|ur, c+|+|+c|º,
ç|+ucor+, +ud co|o|or+ W||| |||udueºº. I|e|e
r+, |e +çeueº|º o| ||e co|puº c+||oºur, redu|·
|o||+º|or+, +ud c+|c|||c+||ou o| ||e |+|\. \eu|+|
|e|+|d+||ou |º |+|e, |oWe.e|. ||||oº+|cor+ o| ||e
j+W, o.+||+u ||||or+º, |e|+|or+º, +ud c,º|+deuo·
r+º |+.e |eeu |epo||ed.
'|·¤ |-º·¤¤º +|e ºr+||, p|upo|u| |o |+|çe| uodu|+|
BCCº (||ç. ¹¹·25), |u| '|eçu|+|,¨ uodu|+|, u|ce|+|·
|uç, +ud ºc|e|oº|uç BCCº +|ºo occu|. Iuro|º ou
||e e,e||dº, +\|||+e, +ud uec| |eud |o |e peduucu·
|+|ed +ud +|e o||eu º,rre|||c ou ||e |+ce. I|e|e
+|e c|+|+c|e||º||c p+|rop|+u|+| |eº|ouº, W||c|
+|e p|eºeu| |u 50° +ud +|e ºr+|| p||º ||+| +|e
p|upo|u| |o ºe.e|+| r||||re|e|º |u º|/e +ud ¹ rr
deep (||ç. ¹¹·2o).
I|e º|çu|||c+uce o| ||e º,ud|ore |º ||+| + |+|çe
uur|e| o| º||u c+uce|º c|e+|e + |||e||re p|o||er
o| .|ç||+uce. I|e ru|||p|e e\c|º|ouº c+u c+uºe +
couº|de|+||e +rouu| o| ºc+|||uç (||ç. ¹¹·25). I|e
|uro|º cou||uue |||ouç|ou| |||e, +ud ||e p+||eu|
ruº| |e |o||oWed c+|e|u||,.
',¤¤¤,¤º. Co|||u º,ud|ore, ue.o|d |+º+| ce||
c+|c|uor+ º,ud|ore.
8ASAI C£II N£vüS S¥N0k0M£ (8CNS) |C|·9 . ¹1!.0
IMAC£ 11-2 8asa| ce|| carcìooma:
predì|ectìoo sìtes |o|º |ud|c+|e
ºupe|||c|+| ru|||ceu|||c BCCº.
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 295
FICük£ 11-25 8asa| ce||
oevus syodrome: sma|| basa|
ce|| carcìoomas 'r+|| |edd|º|
p+pu|+| |eº|ouº +|e d|ºpe|ºed o.e|
||e eu|||e |+ce. A|| o| ||eºe |ep|e·
ºeu| ºr+|| BCCº. |o|e couº|de|+||e
ºc+|||uç ||or |ero.+| o| p|e.|ouº
|eº|ouº. |o|e +|ºo ||ou|+| |oºº|uç
+ud º||+||ºruº.
FICük£ 11-24 8asa| ce|| carcìooma, pì¿-
meoted  A uodu|e W||| |||eçu|+| |o|de|º +ud
.+||eç+||ou o| re|+u|u |ueº, e+º||, cou|uºed W||| +
r+||çu+u| re|+uor+. |e+|u|eº |ud|c+||uç BCC +|e
||e +|e+º o| ||+uº|uceuc, +ud ºu||+ce |e|+uç|ec|+º|+.
 Au |||eçu|+| p||c|·||+c| p|+que W||| + ceu||+|
+|e+ o| |eç|eºº|ou. I||º p|çreu|ed BCC |º c||u|c+||,
|ud|º||uçu|º|+||e ||or ºupe|||c|+| ºp|e+d|uç re|+·
uor+. Corp+|e W||| ||ç. ¹2·¹0C.
FICük£ 11-26 8asa| ce|| oevus syodrome:
pa|mar pìts |+|r+| ºu||+ce o| |+ud º|oW|uç
¹· |o 2·rr, º|+|p|, r+|ç|u+|ed, dep|eººed |eº|ouº, |.e.,
p+|r+| p||º.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 296
C+|c|uor+º o| ||e ecc||ue ºWe+| ç|+ud +|e |+|e
+ud |uc|ude ecc||ue po|oc+|c|uor+, º,||uço|d ec·
c||ue c+|c|uor+, ruc|uouº c+|c|uor+, +ud c|e+|
ce|| ecc||ue c+|c|uor+.
C+|c|uor+º o| ||e +poc||ue ç|+udº +|e +|ºo |+|e,
+||º|uç |u +\|||+e, u|pp|eº, .u|.+, +ud e,e||dº.
C+|c|uor+º o| ||e ºe|+ceouº ç|+udº +|e equ+||,
|+|e, roº| corrou|, +||º|uç ou ||e e,e||dº.
I|eºe |eº|ouº +|e c||u|c+||, |ud|º||uçu|º|+||e ||or
o||e| c+|c|uor+º +ud +|e uºu+||, ro|e +çç|eºº|.e
||+u o||e| |u.+º|.e cu|+ueouº 'CCº.
MAIICNANI AFF£N0AC£ IüM0kS |C|·9 . ¹1!.0
\e||e| ce|| c+|c|uor+ (\CC) (cu|+ueouº ueu·
|oeudoc||ue |uro|) |º + |+|e r+||çu+u| ºo||d
|uro| ||ouç|| |o |e de||.ed ||or + ºpec|+||/ed
ep|||e||+| ce||, ||e \e||e| ce||. || |º + uou|e|+||/|uç,
'c|e+|¨ ce|| p|eºeu| |u ||e |+º+| ce|| |+,e| o| ||e
ep|de|r|º, ||ee |u ||e de|r|º, +ud +|ouud |+||
|o|||c|eº +º ||e |+|| d|º| o| ||u|uº.
\CC occu|º +|roº| e\c|uº|.e|, |u W|||e peop|e.
\CC |º ¹0 |o !0 ||reº +º corrou |u |rruuoºup·
p|eººed p+||eu|º +º |u uou|rruuoºupp|eººed
p+||eu|º.
I|e e||o|oç, |º uu|uoWu |u| r+, |e |e|+|ed |o
c||ou|c u\k d+r+çe. |o|,or+ .||uº |+º |eeu
|ouud |u 30° o| \CC.
I|e |uro| r+, |e ºo|||+|, o| ru|||p|e +ud occu|º
ou ||e |e+d +ud ou ||e e\||er|||eº.
I|e|e |º + ||ç| |+|e o| |ecu||euce |o||oW|uç
e\c|º|ou, |u|, ro|e |rpo||+u|, || ºp|e+dº |o ||e
|eç|ou+| |,rp| uodeº |u > 50° o| p+||eu|º +ud |º
d|ººer|u+|ed |o ||e .|ºce|+ +ud C|'.
\CC p|eºeu|º +º + cu|+ueouº |o ºu|cu|+ue·
ouº p+pu|e, uodu|e, o| |uro| (0.5-5 cr) (||çº.
¹¹·21, ¹¹·23, !o·2!), W||c| |º p|u|, |ed |o .|o|e|
o| |edd|º|·||oWu, dore·º|+ped, +ud uºu+||,
ºo|||+|,. I|e o.e||,|uç º||u |º |u|+c|, |u| |+|çe|
|eº|ouº r+, u|ce|+|e.
I|e, ç|oW |+p|d|, +ud uºu+||, occu| |u pe|ºouº
> 50 ,e+|º.
|e|r+|op+||o|oç, º|oWº uodu|+| o| d|||uºe p+|·
|e|uº o| +çç|eç+|ed, deep|, ||ue º|+|u|uç, ºr+||
|+º+|o|d o| |,rp|or+·|||e·|oo||uç ce||º ||+| c+u
+|ºo |e +||+uçed |u º|ee|º |o|r|uç ueº|º, co|dº,
+ud ||+|ecu|+e.
|rruuoc,|oc|er|º||, º|oWº c,|o|e|+||u +ud
ueu|o|||+reu| r+||e|º, c||oroç|+u|u A, +ud
ueu|ou·ºpec|||c euo|+ºe, e|ec||ou r|c|oºcop, |e·
.e+|º ||e c|+|+c|e||º||c o|ç+ue||eº.
I|e+|reu| |º |, e\c|º|ou o| \o| ºu|çe|,, +ud
ºeu||ue| uode ||opº, o| p|op|,|+c||c |eç|ou+|
uode d|ººec||ou |º +d.oc+|ed |ec+uºe o| ||e ||ç|
|+|e o| |eç|ou+| re|+º|+ºeº. k+d|+||ou ||e|+p, |o
º||e o| \CC +ud |eç|ou+| || |º ç|.eu |u roº| c+ºeº
e\cep| |o| .e|, ºr+|| |eº|ouº.
kecu||euce |+|eº +|e ||ç|, |u oue ºe||eº, e.eu
W|||ou| + |oc+| |ecu||euce, +|ou| o0° o| p+||eu|º
de.e|oped |eç|ou+| uode re|+º|+ºeº, +º d|d 3o°
o| ||oºe p+||eu|º W||| + |oc+| |ecu||euce. ||oçuo·
º|º |º çu+|ded.
M£kk£I C£II CAkCIN0MA |C|·9 . ¹1!.0
°
|C|·¹0 . C++.\32+1/!
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 29T
FICük£ 11-2T Merke| ce|| car-
cìooma A ºr+|| .|o|+ceouº uodu|e
+|o.e ||e p|uu+ ||+| |+d |eeu p|eº·
eu| |o| +|ou| 2 Wee|º. 'eu||ue| |,rp|
uode ||opº, |e.e+|ed re|+º|+º|º o|
ueu|oeudoc||ue c+|c|uor+. A|ºo uo|e
+c||u|c |e|+|oºeº ou ||e |e||\ +ud
couc|+.
FICük£ 11-28 Merke| ce|| carcìooma  A |+|e|, uo||ce+||e o·rr º||ç|||, de|r+| uodu|e |e|oW ||e
|+||||ue ||+| |+d |eeu p|eºeu| |o| +|ou| o Wee|º. ||e+u||cu|+| |,rp| uode re|+º|+º|º W+º +|ºo p|eºeu|.  A
.|o|+ceouº de|r+| uodu|e, ! cr |u d|+re|e| ou ||e |o|e+|r o| + o0·,e+|·o|d r+u. I|e|e W+º re|+º|+º|º |o ||e
+\|||+|, |,rp| uodeº.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 298
A |+|e, |oc+||, +çç|eºº|.e |uro|, º|oW ç|oW|uç,
|u|||+||, o||eu r|º|u|e|p|e|ed +º + ºc+|.
||'| |º + |||r |udu|+|ed p|+que, º||u·co|o|ed |o
|ed·||oWu W||| e\op|,||c uodu|eº (||ç. ¹¹·29).
Au +||op||c .+||+u| r+, |eºer||e º|ce|oº|uç BCC,
ro|p|e+, o| ºc+|.
0ccu|º ou ||e ||uu|, |o||oWed |, ||e e\||er|||eº,
+ud ou|, ¹5° |u ||e |e+d +ud uec| |eç|ou.
|oc+||, +çç|eºº|.e W||| + ||ç| |+|e o| |ecu||euce
+ud |+|e re|+º|+ºeº.
||+çuoº|º |º r+de |, ||º|op+||o|oç,, +ud ||e|+p,
|º W|de ºu|ç|c+| e\c|º|ou. kecu||euceº |eºpoud |o
C|ee.ec.
0£kMAI0FI8k0SAkC0MA Fk0Iü8£kANS (0FSF) |C|·¹0 . \33!!/!
FICük£ 11-29 0ermatoIìbrosarcoma protuberaos Au |||eçu|+| ºc|e|o||c º||u·co|o|ed |o |edd|º| p|+que
o| |uc|e+ºed couº|º|euc, ou ||e |+c| o| + +0·,e+|·o|d r+|e. 0u ||e |oWe| r+|ç|u ||e|e |º + |edd|º| uodu|e |ep|e·
ºeu||uç e\op|,||c ç|oW||. I||º |eº|ou ueedº |o |e e\c|ºed W||| |+|çe r+|ç|uº |o p|e.eu| + |ecu||euce.
S£CII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 299
A uo| ºo |+|e |+p|d|, ç|oW|uç |uro| o| |u|e|red|·
+|e r+||çu+u| po|eu||+|.
A|/ |º +u +º,rp|or+||c, ºo|||+|, p+pu|e, uodu|e,
o| p|+que o||eu |eºer|||uç +u 'CC o| BCC
|u|||+||,.
0ccu|º |u ºuu·d+r+çed º||u o| o|de| p+||eu|º
eºpec|+||, ou |o|e|e+d, ºc+|p. uoºe, +ud e+|º
(||ç. ¹¹·!0).
I|e+|reu| |º ºu|ç|c+|.
AI¥FICAI FI8k0SAkC0MA (AFX) |C|·9 . ¹1!.0
FICük£ 11-30 Atypìca| Iìbroxaothoma I||º |º + 51·,e+|·o|d r+|e W||| de|r+|o|e||oº|º +ud + ||º|o|, o|
ºo|+| |e|+|oºeº, |u.+º|.e +ud |u º||u ºqu+rouº c+|c|uor+, +ud |+º+| ce|| c+|c|uor+. I||º uodu|e ou ||e .e||e\
W+º c||u|c+||, +|,p|c+| |o| e|||e| |+º+| ce|| c+|c|uor+ o| ºqu+rouº ce|| c+|c|uor+, ||º|op+||o|oç, |e.e+|ed +|,p|c+|
||||o\+u||or+.
300
S E C I | 0 N 1 2
||ecu|ºo|º o| re|+uor+ +|e |eº|ouº ||+| +|e
|eu|çu pe| ºe |u| |+.e ||e po|eu||+| o| |u|u|uç
r+||çu+u| +ud ||uº ç|.|uç ||ºe |o re|+uor+.
IWo ºuc| eu||||eº +|e |ecoçu|/ed. (¹) d,ºp|+º·
||c ue.ore|+uoc,||c ue.|, +ud (2) couçeu||+|
ue.ore|+uoc,||c ue.|.
Fk£CükS0kS 0F CüIAN£0üS M£IAN0MA
£FI0£MI0I0C¥
A¿e oI 0oset Childien and adults.
Freva|eoce || aie piesent in 5% of the geneial
white population. They occui in almost eveiy
patient with familial cutaneous melanoma and
in 30-50% of patients with spoiadic nonfamil-
ial piimaiy melanomas of the skin.
Sex Equal in males and females.
kace White peisons. Data on peisons with
biown oi black skin aie not available; DN aie
iaiely seen in the Japanese population.
Iraosmìssìoo Autosomal dominant.
FAIh0C£N£SIS
Multiple loci have been implicated in familial
melanoma/DN syndiome. It is assumed than an
abnoimal clone of melanocytes can be activated
by exposuie to sunlight. Immunosuppiessed
M£IAN0MA Fk£CükS0kS
AN0 FkIMAk¥ CüIAN£0üS
M£IAN0MA
|,ºp|+º||c re|+uoc,||c ue.| (||) +|e + ºpec|+|
|,pe o| +cqu||ed, c||curºc|||ed, p|çreu|ed
|eº|ouº ||+| |ep|eºeu| d|ºo|de|ed p|o|||e|+||ouº o|
.+||+||, +|,p|c+| re|+uoc,|eº.
|| +||ºe de uo.o o| +º p+|| o| + corpouud
re|+uoc,||c ue.uº.
|| +|e c||u|c+||, d|º||uc||.e ||or corrou +c·
qu||ed ue.|. |+|çe| +ud ro|e .+||eç+|ed |u co|o|,
+º,rre|||c |u ou|||ue, |||eçu|+| |o|de|º, ||e, +|ºo
|+.e c|+|+c|e||º||c ||º|o|oç|c |e+|u|eº.
|| +|e |eç+|ded +º po|eu||+| p|ecu|ºo|º o| ºupe|·
||c|+| ºp|e+d|uç re|+uor+ +ud +|ºo +º r+||e|º o|
pe|ºouº +| ||º| |o| de.e|op|uç p||r+|, r+||çu+u|
re|+uor+ o| ||e º||u, e|||e| W||||u ||e || o| ou
'uo|r+|¨ º||u.
|| occu| e|||e| ºpo|+d|c+||, o| |u ||e cou|e\| o|
||e |c¤·|·c| || º,¤!·¤¤- . ||ud|edº W||| |+r|||+|
ru|||p|e || +ud re|+uor+º (|o|re||, |A\\\,
o| B·K ro|e º,ud|ore).
',uou,rº. +|,p|c+| re|+uoc,||c ue.uº, d+|| ue.uº
0¥SFIASIIC M£IAN0C¥IIC N£vüS |C|·9 . 2!3.2
°
|C|·¹0 . |+3·5
patients (ienal tiansplantation) with DN have
a highei incidence of melanoma. DN favoi the
exposed aieas of the skin, and this may be ie-
lated to the degiee of sun exposuie.
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos DN usually aiise latei in
childhood than common acquiied nevomelano-
cytic nevi (NMN), appeaiing fiist in late child-
hood, just befoie pubeity. New lesions continue
to develop ovei many yeais in affected peisons;
in contiast, common acquiied NMN do not ap-
peai aftei middle age and disappeai entiiely in
oldei peisons. Also, wheieas common NMN aie
usually in a ioughly compaiable stage of devel-
opment in a given body iegion (e.g., junctional,
compound, deimal), DN appeai °out of step,"
e.g., a mix of laige and small, flat and iaised, tan
and veiy daik lesions (Fig. 12-1À).
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 301
IA8I£ 12-1 Comparative |eatures of Common Nevome|anocytic Nevi (NNN), 0ysp|astic Nevi (0N),
and Superficia| Spreadin¿ Ne|anoma (SSN)
hNh 0h SSN
Les|oo F|gs. 9-1 to 9-4) (F|gs. 12-1 aod 12-2) (F|gs. 12-10 aod 12-11)
|ur|e| 'e.e|+| o| r+u, 0ue o| r+u, '|uç|e (¹-2° |+.e
ru|||p|e)
||º||||u||ou \oº||, ||uu|, \oº||, ||uu|, Au,W|e|e |u| p|edor|u+u|
e\||er|||eº e\||er|||eº uppe| |+c|, |eçº
0uºe| C|||d|ood, E+||, +do|eºceuce Au, +çe, roº| |u
+do|eºceuce +du|||ood
I,pe \+cu|eº (juuc||ou+|) \+cu|eº W||| |+|ºed ||+que, |||eçu|+|
|+pu|eº (corpouud, po|||ouº (+º,rre|||c+||,,
de|r+|) r+cu|op+pu|+|)
A Aº,rre||, ',rre||, Aº,rre||, C|e+|e| +º,rre||,
B Bo|de| keçu|+|, We||·de||ued |||eçu|+|, |||· +ud |||eçu|+|, We||·de||ued
We||·de||ued
C Co|o| I+u, ||oWu, d+|| I+u, ||oWu, d+|| I+u, ||oWu, d+|| ||oWu,
||oWu, uu||o|r, ||oWu, ||+c|, p|u|, |ed, ||ue,
o|de||, p+||e|u p|u|, |ed, uo| uu||o|r, W|||e, uºu+||, + r|\,
.+||eç+|ed p+||e|u, ||ç||, .+||eç+|ed,
'|||ed eçç,¨ '|+|çe|o|d¨ ºpo||ed, ºpec||ed p+||e|u
| ||+re|e| <5 rr, |+|e|, <¹0 rr up |o ¹5 rr \oº| >5 rr (|u|, o|
cou|ºe, º|+||º ºr+||e|)
E Eu|+|çereu| '|opº |u +do|eºceuce Cou||uueº |u C|oW|| |u º|/e +| +u,
+du|||ood |u| ||r||ed +çe, uu||r||ed
FICük£ 12-1 0ysp|astìc oevì â. 0.e|.|eW o| ||e |+c| o| + p+||eu| W||| corrou +ud d,ºp|+º||c ue.|. |o|e
+ uur|e| o| |eº|ouº +|e o| d|||e|eu| º|/e +ud co|o|, 'ou| o| º|ep¨. I|e |eº|ou r+||ed |, +u +||oW W+º +u ''\. 8.
|+|çe| r+çu|||c+||ou o| |Wo ||º. |o|e |||eçu|+|||,, .+||eç+||ou o| co|o| W||c| +|e d|||e|eu| |u ||e |Wo |eº|ouº ('ou|
o| º|ep¨). A|ºo, ||e |eº|ouº +|e ¹ cr o| |+|çe| |u d|+re|e|. I|e +||oW deuo|eº + ºe|o|||e|c |e|+|oº|º. I|e ºr+||e|
|eº|ouº +|e corrou |\|.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 302
DN aie thought not to undeigo spontaneous
iegiession at all oi at least much less than com-
mon acquiied NMN.
Frecìpìtatìo¿ Factors Exposuie to sunlight is
iegaided by some as an inducing agent foi DN;
neveitheless, DN aie not infiequently obseived
in completely coveied aieas such as the scalp
and anogenital aieas.
Skìo Symptoms Asymptomatic.
Famì|y hìstory In the familial setting, family
membeis can develop melanoma without the
piesence of DN.
C|ìoìca| Features DN show some of the fea-
tuies of common NMN and some of supeifi-
cial spieading melanoma, so that they occupy
an inteimediaiy position between these two
moiphologies (Table 12-1). No single featuie
is diagnostic; iathei, theie is a constellation of
findings. They aie moie iiiegulai, lightei than
common NMN, usually maculopapulai (DN
have a maculai component); have distinct anJ
indistinct boideis (Figs. 12-1 and 12-2), and a
gieatei complexity of coloi than common nevi
(Figs. 12-1 and 12-2) but less than melanoma.
°Fiied-egg" and °taigeted" types (see Fig. 12-2E
and Table 12-1). Melanoma aiising in an DN
appeais initially as a small papule (often of a
diffeient coloi) oi change in coloi pattein and
massive coloi change within the piecuisoi le-
sion (Fig. 12-3).
0ermoscopy This noninvasive technique al-
lows foi clinical impiovement of diagnostic
accuiacy in DN by >50%. Dígíìa| Jermosto¡y
peimits computeiized follow-up of lesions and
immediate detection of any change ovei time,
indicating developing malignancy.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Hypeiplasia and piolifeia-
tion of melanocytes in a single-file, °lentiginous"
pattein in the basal cell layei eithei as spindle
cells oi as epithelioid cells and as iiiegulai and
dyshesive nests. °Atypical" melanocytes, °biidg-
ing" between iete iidges by melanocytic nests;
spindle-shaped melanocytes oiiented paiallel
to skin suiface. Lamellai fibioplasia and con-
centiic eosinophilic fibiosis (not a constant fea-
tuie). Histologic atypia do not always coiielate
with clinical atypia. DN may aiise in contiguity
with a compound NMN (iaiely, a junctional
nevus) that is centially located, i.e., DN often
have extension of intiaepideimal melanocytic
hypeiplasia beyond the shouldei of the deimal
nevus component; some DN may not have a
deimal nevus component.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
The diagnosis of DN is made by clinical iecog-
nition of typical distinctive lesions (see Table
12-1), and diagnostic accuiacy is consideiably
impioved by deimoscopy. The clinicopatho-
logic coiielations aie now well documented.
Siblings, childien, and paients should also be
examined foi DN once the diagnosis is estab-
lished in a family membei.
0ìIIereotìa| 0ìa¿oosìs Congenital NMN, com-
mon acquiied NMN, supeificial spieading ma-
lignant melanoma, melanoma in situ, lentigo
maligna, Spitz nevus, pigmented basal cell cai-
cinoma.
Assocìatìoo wìth Me|aooma DN aie iegaided
as maikeis foi peisons at iisk foi melanoma
and as piecuisois of supeificial spieading
melanoma. Anatomic association (in conti-
guity) of DN has been obseived in 36% of
spoiadic piimaiy melanomas, in about 70%
of familial piimaiy melanomas, and in 94% of
melanomas with familial melanoma and DN.
IìIetìme kìsks oI 0eve|opìo¿ Frìmary Ma|ì¿oaot
Me|aooma
· Geneial population: 1.2%
· Familial DN syndiome with ìwo blood iela-
tives with melanoma: 100%
· All othei patients with DN: 18%
· The piesence of one DN doubles the iisk foi
development of melanoma; with 10 DN, the
iisk incieases 12-fold.
MANAC£M£NI
Suigical excision of lesions with naiiow mai-
gins. Lasei oi othei types of physical destiuc-
tion should ne·er be used because they do not
peimit histopathologic veiification of diagnosis.
The following guidelines foi selection of lesions
to be excised aie suggested:
· Lesions that aie changing (inciease in size,
change in pigmentation pattein, changes in
shape and/oi boidei); decision is best and
most ieliably made by digital deimoscopy.
· Lesions that cannot be closely followed by
the patient by self-examination (on the scalp,
genitalia, uppei back).
Patients with DN in the familial melanoma set-
ting need to be followed caiefully: in familial
DN, eveiy 3 months; in spoiadic DN, eveiy 6
months to 1 yeai. Seaich foi changes in exist-
ing DN and development of new nevi. Photo-
giaphic follow-up is impoitant, with the tiunk
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 303
FICük£ 12-2 0ysp|astìc oevì â. A |+|çe, uu||o|r|, |+u, .e|, ||+| r+cu|+| o.+| |eº|ou. I|e uo|c|ed |o|de|
ou ||e |e|| +ud ||e º|/e (>¹ cr) +|e ||e ou|, c|||e||+ r+||uç |||º ºuºp|c|ouº o| + ||. 8. I|ouç| |e|+||.e|, º,r·
re|||c |||º |eº|ou |º r+cu|+| +ud p+pu|+| W||| + .+||eç+|ed co|o| +ud re+ºu|eº ¹.5 cr |u d|+re|e|. I|e ºr+||e|
|eº|ouº +|e corrou |\|. C. A ||ç||, +º,rre|||c, |o|| |||· +ud º|+|p|, de||ued r+|ç|u, + uo|c|ed |o|de|, +ud
.+||eç+|ed ||oWu |o ||+c| co|o|. || |º c||u|c+||, |ud|º||uçu|º|+||e ||or +u ''\ (ºee ||çº. ¹2·¹0A, B) |u| W+º ||º|o·
|oç|c+||, + ||. 0 I||º |eº|ou |º +º,rre|||c W||| +u |||eçu|+|, uo|c|ed |o|de|, || |+º + r+cu|+| corpoueu| +ud
+ pe|||, ºu||+ce |u ||e p+pu|+| po|||ou. F. A |e|+||.e|, º,rre|||c º|+|p|, de||ued |eº|ou W||| +u ecceu|||c, ro|e
|e+.||, p|çreu|ed +|e+ (|+|çe|o|d |eº|ou).
â
C
F
8
0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 304
and extiemities; also (1:1) of laigei le-
sions (>6 mm) and all lesions that have
some vaiiegation. Most ieliable method
is digitalized deimoscopy, which should
be available in eveiy pigmented lesion
and melanoma centei. Patients should
be given coloi-illustiated pamphlets
that depict the clinical appeaiance of
DN, malignant melanoma, and com-
mon acquiied NMN. Patients with DN
(familial and nonfamilial) should not
sunbathe and should use sunscieens
when outdoois. They should not use
tanning pailois. Family membeis of
the patient should also be examined
iegulaily.
C|\| +|e p|çreu|ed |eº|ouº o| ||e º||u uºu·
+||, p|eºeu| +| |||||, |+|e .+||e||eº o| C|\| c+u
de.e|op +ud |ecore c||u|c+||, +pp+|eu| du||uç
|u|+uc,.
C|\| r+, |e +u, º|/e ||or .e|, ºr+|| |o .e|,
|+|çe.
C|\| +|e |eu|çu ue.ore|+uoc,||c ueop|+ºrº.
noWe.e|, +|| C|\|, |eç+|d|eºº o| º|/e, r+, |e
p|ecu|ºo|º o| r+||çu+u| re|+uor+.
*
C|+u| C|\C +|e .e|, |+|e.
C0NC£NIIAI N£v0M£IAN0C¥IIC N£vüS (CNMN) |C|·9 . 151.!!
°
|C|·¹0 . |22
£FI0£MI0I0C¥
Freva|eoce Piesent in 1% of white newboins;
the majoiity <3 cm in diametei. Laigei CNMN
aie piesent in 1:2000 to 1:20,000 newboins. Le-
sions 9.9 cm in diametei have a pievalence of
1:20,000, and giant CNMN (occupying a majoi
poition of a majoi anatomic site) occui in
1:500,000 newboins.
A¿e oI 0oset Piesent at biith (congenital).
Some CNMN become visible only aftei biith
(ìarJí·e ), °fading in" as a ielatively laige lesion
ovei a peiiod of weeks.
Sex Equal pievalence in males and females.
kace All iaces.
FAIh0C£N£SIS
Congenital and acquiied nevomelanocytic
nevi aie piesumed to occui as the iesult of a
developmental defect in neuial ciest-deiived
melanoblasts. This defect piobably occuis aftei
10 weeks in uteio but befoie the sixth uteiine
month; the occuiience of the °split" nevus of
the eyelid, i.e., half of the nevus on the uppei
and half on the lowei eyelid, is an indication
that nevomelanocytes migiating fiom the neu-
ial ciest weie in place in this site befoie the
eyelids split (24 weeks).
FICük£ 12-3 SuperIìcìa| spreadìo¿ me|aooma: arìsìo¿
wìthìo a dysp|astìc oevus I|e uppe| d+|| ||oWu po|||ou
W||| + p|u||º| ||r o| |||º |eº|ou |º + d,ºp|+º||c ue.uº, ||e .+||e·
ç+|ed ||ue·||+c| +ud p|u| p|+que |u ||e |oWe| |+|| o| ||e |eº|ou
|º ||e ºupe|||c|+| ºp|e+d|uç re|+uor+ (0.9·rr |||c|ueºº) +||º·
|uç W||||u ||e d,ºp|+º||c ue.uº.
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 305
Sma|| aod Iar¿e CNMN CNMN have a iathei
wide iange of clinical featuies, but the following
aie typical (Figs. 12-4 and 12-5): CNMN usually
distoit the skin suiface to some degiee and aie
theiefoie a plaque with oi without coaise teimi-
nal daik biown oi black haiis (haii giowth has
a delayed onset) (Figs. 12-4B, 12-5B). Shaiply
demaicated (Fig. 12-4) oi meiging impeicepti-
bly with suiiounding skin; iegulai oi iiiegulai
contouis. Laige lesions may be °woimy" oi soft
(Fig. 12-5À), iaiely fiim (desmoplastic type).
Skin suiface smooth oi °pebbly," mamillated,
iugose, ceiebiifoim, bulbous, tubeious, oi lob-
ulai (Fig. 12-5À). These suiface changes aie
obseived moie fiequently in lesions that extend
deep into the ieticulai deimis.
Cv|vr Light oi daik biown. With deimos-
copy a fine speckling of a daikei hue with a
lightei suiiounding biown hue is seen; often
the pigmentation is folliculai. °Halo" CNMN
aie iaie.
SIze Small (Fig. 12-4), laige (>20 cm), oi giant
(Fig. 12-5). °Acquiied" nevomelanocytic nevi >
1.5 cm in diametei should be iegaided as piob-
ably taidive CNMN oi they iepiesent DN.
Shupe Oval oi iound.
DIstrIhutIvn v] LesIvns Isolated, disciete le-
sion in any site. Fewei than 5% of CNMN aie
multiple. Multiple lesions aie moie common in
association with laige CNMN. Numeious small
CNMN occui in patients with giant CNMN, in
whom theie may be numeious small CNMN on
the tiunk and extiemities away fiom the site of
the giant CNMN (Fig. 12-5À).
very Iar¿e ("Cìaot") CNMN
Giant CNMN of the head and neck may be as-
sociated with involvement of the leptomeninges
with the same pathologic piocess; this piesenta-
tion may be asymptomatic oi manifested by
seizuies, focal neuiologic defects, oi obstiuc-
tive hydiocephalus. Giant CNMN is usually a
plaque with suiface distoition, coveiing entiie
segments of the tiunk, extiemities, head, oi
neck (Fig. 12-5).
Me|aooma ìo CNMN
A papule oi nodule aiises within CNMN
(Fig. 12-6). Often melanoma aiises in deimal
oi subcutaneous nevomelanocytes and can be
fai advanced when detected.
0IFF£k£NIIAI 0IACN0SIS
Common acquiied NMN, DN, congenital blue
nevus, nevus spilus, Beckei nevus, pigmented
epideimal nevi, and café-au-lait macules should
be consideied in the diffeiential diagnosis of
CNMN. Small CNMN aie viitually indistin-
guishable clinically fiom common acquiied
NMN except foi size, and lesions >1.5 cm
may be piesumed to be eithei taidive CNMN
oi DN.
FICük£ 12-4 Coo¿eoìta| oevome|aoocytìc oevus â. 'r+||, .+||eç+|ed ||oWu p|+que ou ||e uoºe. I|e
|eº|ou W+º p|eºeu| +| |||||. |o|e ||+| |eº|ou |º |+||,. 8. Couçeu||+| ue.ore|+uoc,||c ue.uº, |u|e|red|+|e º|/e.
'|+|p|, der+|c+|ed c|oco|+|e·||oWu p|+que W||| º|+|p|, de||ued |o|de|º |u +u |u|+u|. w||| |uc|e+º|uç +çe,
|eº|ouº r+, |ecore e|e.+|ed +ud |+||, +ud .e|, d|ºc|e|e |+|||ueºº |º +|ºo uo|ed |u |||º |eº|ou.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 306
IA80kAI0k¥ £XAMINAII0N
hìstopatho|o¿y Nevomelanocytes occui as
well-oideied clusteis ( ì|eques ) in the epideimis
and in the deimis as sheets, nests, oi coids. À
Jí[[use ín[í|ìraìíon o[ sìranJs o[ ne·ome|ano-
tyìes ín ì|e |ower one-ì|írJ o[ ì|e reìítu|ar Jer-
mís anJ su|tuìís ís, w|en ¡resenì, quíìe s¡etí[ít
[or CNMN . In laige and giant CNMN, the
nevomelanocytes may extend into the muscle,
bone, duia matei, and cianium.
C0ükS£ AN0 Fk0CN0SIS
By definition, CNMN appeai at biith, but
CNMN may aiise duiing infancy (ìarJí·e
CNMN). The life histoiy of CNMN is not
documented, but CNMN have been obseived
in eldeily peisons, an age when the common
acquiied NMN have disappeaied.
Large or gíanì CNMN. The lifetime iisk foi
development of melanoma in laige CNMN
has been estimated to be at least 6.3%. In 50%
of patients who develop melanoma in laige
CNMN, the diagnosis is made between the ages
of 3 and 5 yeais. Melanoma that develops in a
laige CNMN has a pooi piognosis because it is
detected late.
Sma|| CNMN : The lifetime iisk of develop-
ing malignant melanoma is 1-5%. Based on the
detection of congenital nevi in association with
melanoma by means of histology and a caieful
histoiy, a significantly incieased iisk is appaient
foi developing melanoma in peisons with small
CNMN. This iisk is as high as 21-fold based on
histoiy and 3- to 10-fold based on histology.
The expected association of small CNMN and
melanoma is <1:171,000 based on chance alone.
Nonetheless, small CNMN should be consid-
eied foi piophylactic excision at pubeity if theie
aie no atypical featuies (vaiiegated coloi and
iiiegulai boideis); small CNMN with
atypical featuies should be excised im-
mediately.
MANAC£M£NI
Sur¿ìca| £xcìsìoo The only acceptable
method. Sma|| anJ |arge CNMN. Exci-
sion, with full-thickness skin giaft, if
iequiied; swing flaps, tissue expandeis
foi laige lesions. Cíanì CNMN. Risk of
development of melanoma is significant
even in the fiist 3 to 5 yeais of age, and
thus giant CNMN should be iemoved
as soon as possible. Individual consid-
eiations aie necessaiy (size, location,
degiee of loss of function, oi amount
of mutilation). New suigical techniques
utilizing the patient's own noimal skin
giown in tissue cultuie can now be
used to facilitate iemoval of veiy laige
CNMN. Also, tissue expandeis can be
used.
FICük£ 12-5 Cìaot coo¿eoìta|
oevome|aoocytìc oevus â. |u |||º |+|,
||e |eº|ou |u.o|.eº ||e r+jo|||, o| ||e º||u,
W||| corp|e|e |ep|+cereu| o| uo|r+| º||u ou
||e |+c| +ud ru|||p|e ºr+||e| C|\| ou ||e
|u||oc|º +ud |||ç|º. I|e|e |º |,pe||||c|oº|º |u
||e uppe| po|||ou. \e|+uor+ de.e|op|uç |u +
ç|+u| C|\| |º d||||cu|| |o d|+çuoºe e+||, |u +
ºe|||uç o| ºuc| ||ç||, +|uo|r+| ||ººue.
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 30T
FICük£ 12-5 Cìaot coo¿eoìta| oevome|aoocytìc oevus (Cootìoued ) 8. C|+u| C|\| |u + 52·,e+|·o|d
r+|e. I|e r+ºº|.e |,pe||||c|oº|º +ddº |o ||e d||||cu||, o| d|+çuoº|uç re|+uor+ |u |||º |eº|ou +| |||º º|+çe.
FICük£ 12-6 Me|aooma: arìsìo¿ ìo sma|| CNMN A ||+c| p|+que ou ||e |||ç| o| + !o·,e+|·o|d |er+|e,
W||c| |+º |eeu p|eºeu| º|uce |||||. keceu||, + º||ç|||, |eºº p|çreu|ed e\ceu|||c uodu|e |+d +ppe+|ed |u |||º
|eº|ou. I||º |º + re|+uor+.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 308
CIASSIFICAII0N 0F M£IAN0MA
I De novo melanoma
A. Melanoma in situ (MIS)
B. Lentigo maligna melanoma (LMM)
C. Supeificial spieading melanoma (SSM)
D. Nodulai melanoma (NM)
E. Acial-lentiginous melanoma (ALM)
F. Melanoma of the mucous membianes
G. Desmoplastic melanoma
II Melanoma aiising fiom piecuisois
A. Melanoma aiising in dysplastic
nevomelanocytic nevi
B. Melanoma aiising in congenital
nevomelanocytic nevi
C. Melanoma aiising in common NMN
F0ük IMF0kIANI M£SSAC£S C0NC£kNINC
CüIAN£0üS M£IAN0MA
1. Me|aooma oI the Skìo Is Approachìo¿
£pìdemìc Froportìoos
Melanoma is a common malignancy and its inci-
dence is on the iise. In the United States the
lifetime iisk of invasive melanoma developing was
only 1 in 1500 in 1935; in 1992 it was 1 in 105, in
2002 it was 1 in 75, and in 2010 it is estimated that
it will be 1 in 50. In 2008, 60,000 cases of melanoma
weie iecoided and theie weie 8000 deaths fiom
melanoma in the United States; the numbei of
melanomas in the United States continues to in-
ciease by 7% pei yeai. Cutaneous melanoma cui-
iently iepiesents 5% of newly diagnosed cancei in
men and 6% in women. It is the leading fatal illness
aiising in the skin and is iesponsible foi 80% of
deaths fiom skin cancei. U.S. cancei statistics show
that melanoma had the second highest moitality
iate inciease among men 65 yeais old. On the
othei hand, deaths fiom melanoma occui at a
youngei age than deaths fiom most othei canceis,
and melanoma is among the most common types
of cancei in young adults.
2. £ar|y keco¿oìtìoo aod £xcìsìoo oI Frìmary
Me|aooma kesu|t ìo vìrtua| Cure
Cuiient cutaneous melanoma education
stiesses the detection of eaily melanoma, with
Cu|+ueouº re|+uor+ |º ||e roº| r+||çu+u|
|uro| o| ||e º||u. \e|+uor+ +||ºeº ||or ||e
r+||çu+u| ||+uº|o|r+||ou o| re|+uoc,|eº +|
||e de|r+|·ep|de|r+| juuc||ou o| ||or ||e
ue.ore|+uoc,|eº o| d,ºp|+º||c re|+uoc,||c
ue.| o| C|\| ||+| |ecore |u.+º|.e +ud re·
|+º|+º|/e +||e| .+||ouº ||re |u|e|.+|º.
CüIAN£0üS M£IAN0MA |C|·9 . ¹12
°
|C|·¹0 . C+! 
high cuie iates aftei suigical excision. Of all the
canceis, melanoma of the skin is the most ie-
waiding foi detection of eaily cuiable piimaiy
tumois, theieby pieventing metastatic disease
and death. Eaily accessability to physicians is
especially impoitant because cuiability is di-
iectly ielated to size and depth of invasion of
the tumoi. At the piesent time, the most ciitical
tool foi conqueiing this disease is, theiefoie,
the identification of eaily °thin" melanomas by
clinical examination. Total skin examination foi
melanoma and its piecuisois should be done
ioutinely.
About 30% of melanomas aiise in a pieexist-
ing melanocytic lesion; 70% aiise in noimal
skin. Almost all melanomas show an initial
iadial giowth phase followed by a subsequent
veitical giowth phase. Since metastasis occuis
only infiequently duiing the iadial giowth
phase, detection of eaily melanomas (i.e., °thin"
melanomas) duiing this phase is essential.
Theie is the paiadox that even with a iising
moitality iate, theie has been an encouiag-
ing impiovement in the oveiall piognosis of
melanoma with veiy high 5-yeai suivival iates
(appioaching 98%) foi thin (<0.75 mm) pii-
maiy melanoma and an 83% iate foi all stages.
The favoiable piognosis is entiiely attiibutable
to eaily detection.
3. A|| Fhysìcìaos aod Nurses have the
kespoosìbì|ìty oI 0etectìo¿ £ar|y Me|aooma
Eaily detection of piimaiy melanoma assuies
incieased suivival; advanced piimaiy melanoma
has a pooi piognosis and suivival. The suivival
iate plummets when theie is iegional metas-
tasis to lymph nodes. The seiiousness of this
disease thus places the iesponsibility on the
health caie piovidei in the pivotal iole: not to
oveilook pigmented lesions. This is especially
tiue foi the piimaiy caie physician, the nuise,
the physical theiapist, oi a health caie piovidei
who sees the total skin of the body. Theiefoie,
it is iecommended that in clinical piactice, no
mattei what is the piesenting complaint, total
examination of the body should be iequested
of all nonpigmented (i.e., white) patients at
the time of the fiist encountei and that all
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 309
IA8I£ 12-2 |ittpatrick NNR|SK
A moemoo|c dev|ce Ior promot|og me|aooma r|sk awareoess amoog phys|c|aos aod pat|eots. £ach |etter
represeots ooe oI the major r|sk Iactors Ior me|aooma oI the sk|o.
M \o|eº. +|,p|c+| (d,ºp|+º||c ue.uº) (>5)
M \o|eº. corrou ro|eº (uure|ouº, >50)
k ked |+|| +ud ||ec|||uç (o||eu ||eºe pe|ºouº |+.e |eW o| uo ro|eº)
I |u+|||||, |o |+u. º||u p|o|o|,peº | +ud ||
S 'uu|u|u. ºe.e|e ºuu|u|u eºpec|+||, |e|o|e +çe ¹+
k K|ud|ed. |+r||, ||º|o|, o| re|+uor+
IA8I£ 12-3 Risk |actors for the 0eve|opment of Ne|anoma
· Ceue||c r+||e|º (C|||2c ru|+||ou)
· '||u |,pe |/||
· |+r||, ||º|o|, o| d,ºp|+º||c ue.| o| re|+uor+
· |e|ºou+| ||º|o|, o| re|+uor+
· u|||+.|o|e| |||+d|+||ou, p+|||cu|+||, ºuu|u|uº du||uç c|||d|ood +ud |u|e|r|||eu| |u|u|uç e\poºu|eº
· |ur|e| (>50) +ud º|/e (>5 rr) o| re|+uoc,||c ue.|
· Couçeu||+| ue.|
· |ur|e| o| d,ºp|+º||c ue.| (>5)
· |,ºp|+º||c re|+uoc,||c ue.uº º,ud|ore
body iegions, including the scalp, toewebs, and
oiifices (mouth, anus, vulva), be examined. It is
helpful to question patients accoiding to a mne-
monic list of melanoma iisk (Table 12-2).
4. £xamìoatìoo oI A|| Acquìred Fì¿meoted
Iesìoos Accordìo¿ to the A8C0£ ku|e
This iule analyzes pigmented lesions accoiding
to symmetiy, boidei, coloi, diametei, giowth
and elevation (see page 310). While it does not
apply to all types of melanoma it peimits dif-
feiential diagnostic sepaiation of most melano-
mas fiom common nevi and othei pigmented
lesions.
£II0I0C¥ AN0 FAIh0C£N£SIS
The etiology and pathogenesis of cutaneous
melanoma aie unknown. Epidemiologic studies
demonstiate a iole foi genetic piedisposition
and sun exposuie in melanoma development.
The majoi genes involved in melanoma devel-
opment ieside on chiomosome 9p21. 25 to 40%
of membeis of melanoma-pione families have
mutations in cyclin-dependent kinase inhibitoi
2A (CDKN2À) and a few families in cyclin-
dependent kinase 4 (CDK4). These aie tumoi
suppiessoi genes that piovide a iational basis
foi the link to susceptibility to melanoma.
Theie is convincing evidence fiom epidemio-
logic studies that exposuie to solai iadiation
is the majoi cause of cutaneous melanoma.
Cutaneous melanoma is a gieatei pioblem in
light-skinned whites (skin types I and II), and
sunbuins duiing childhood and inteimittent
buining exposuie in faii skin seem to have a
highei impact than cumulative UV exposuie
ovei time. Othei piedisposing and iisk factois
aie the piesence of piecuisoi lesions (dysplastic
melanocytic nevi and congenital nevomelano-
cytic nevi) and a family histoiy of melanoma
in paients, childien oi siblings. Risk factois foi
melanoma aie listed in Table 12-3.
M£IAN0MA Ck0WIh FAII£kNS
Almost all melanomas show an initial iadial
giowth phase followed by a subsequent veitical
giowth phase. RaJía| growì| ¡|ase iefeis to a
mostly intiaepideimal, pieinvasive, oi minimally
invasive giowth pattein; ·erìíta| growì| iefeis to
giowth into the deimis and thus into the vicin-
ity of vessels that seive as avenues foi metas-
tasis. Since most melanomas pioduce melanin
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 310
pigment, even pieinvasive melanomas in theii
iadial giowth phase aie clinically detectable by
theii coloi patteins. The piognostic diffeience
among the clinical types of melanoma ielates
mainly to the duiation of the iadial giowth
phase, which may last fiom yeais to decades in
lentigo maligna melanoma, fiom months to 2
yeais in supeificial spieading melanoma, and 6
months oi less in nodulai melanoma.
0AIA AN0 FACIS
· Melanoma iepiesents 5% of all canceis by
incidence in males and 6% in females.
· Numbei of new cases in the United States in
2008: 62,000.
· U.S. lifetime iisk of developing invasive
melanoma in 2010: 1/50.
· New melanoma deaths in United States, 2008:
8400.
· Most fiequent sites
· Whites
Male: back, uppei extiemities.
Female: back, lowei legs.
· Blacks and Asians: soles, mucous membianes,
palms, nail beds.
· Fiequency of melanoma by type of tumoi: su-
peificial spieading melanoma: 70%; nodulai
melanoma: 15%; lentigo maligna melanoma:
5%; acial and unclassified melanoma: 10%.
M£IAN0MA k£C0CNIII0N
Sìx Sì¿os oI Ma|ì¿oaot Me|aooma (A8C0£ ku|e)
A Àsymmeìry in shape-one-half unlike the
othei half.
B BorJer is iiiegulai-edges iiiegulaily scal-
loped, notched, shaiply defined.
C Co|or is not unifoim; mottled-haphazaid
display of colois; all shades of biown,
black, giay, ied, and white.
D Díameìer is usually laige-gieatei than
the tip of a pencil eiasei (6.0 mm); oth-
eis use D foi °ugly duckling" sign: lesion
is diffeient with iespect to change in size,
shape, coloi.
E E|e·aìíon is almost always piesent and is
iiiegulai-suiface distoition is assessed
by side-lighting. Melanoma in situ and
acial lentiginous lesions initially maculai;
otheis use E foi En|argemenì- a histoiy
of an inciease in the size of lesion is one
of the most impoitant signs of malignant
melanoma.
0L|h|0AL P8£S£hTAT|0hS 0F N£LAh0NA
The clinical chaiacteiistics of the foui ma-
joi types of melanoma aie summaiized in
Table 12-4. Also discussed in this section aie
melanoma in situ and desmoplastic melanoma.
IA8I£ 12-4 |our Najor Iypes of Ne|anoma
Type Freg0eocy, % S|te 8ad|a| 6rowth Vert|ca| 6rowth
'upe|||c|+| 10 Au, º||e, |oWe| \ou||º |o |e|+,ed
ºp|e+d|uç e\||er|||eº, 2 ,e+|º
||uu|
|odu|+| ¹5 Au, º||e, ||uu|, |o c||u|c+||, |rred|+|e
|e+d, uec| pe|cep||||e
|+d|+| ç|oW||
|eu||ço 5 |+ce, uec|, do|º+ \e+|º \uc| de|+,ed
r+||çu+ o| |+udº
re|+uor+
Ac|+| 5-¹0 |+|rº, ºo|eº, \ou||º |o E+||, |u|
|eu||ç|uouº ºu|uuçu+| ,e+|º |ecoçu|||ou
re|+uor+ de|+,ed
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 311
I|e c||u|c+| |e+|u|eº o| \|' +|e uo| +|W+,º
c|e+||, p|eºeu|ed. \|' |º p||r+|||, + ||º·
|op+||o|oç|c de||u|||ou, +ud ||e |e|r |º uºed
W|eu re|+uor+ ce||º +|e cou||ued |o ||e
ep|de|r|º, +|o.e ||e |+ºereu| rer||+ue,
|+º||+| re|+uoc,||c +|,p|+, |,pe|p|+º|+, +ud
ºp|e+d e|||e| occu| |u º|uç|e·|||e +||çureu|
+|ouç ||e |+º+| rer||+ue o| +|e d|º||||u|ed
|||ouç|ou| ||e ep|de|r|º (p+çe|o|d ºp|e+d).
E.e|, re|+uor+ º|+||º +º +u |u º||u |eº|ou, |u|
\|' |º c||u|c+||, d|+çuoº+||e ou|, W|eu ||e
|+d|+| ç|oW|| p|+ºe |º |ouç euouç| |o| || |o |e·
core .|ºu+||, de|ec|+||e. 'uc| |eº|ouº +|e ||+|,
W||||u ||e |e.e| o| ||e º||u, +ud ||uº + ¤c:±|-
(||ç. ¹2·1) o| + r+cu|e W||| |+|e|, pe|cep||||e
e|e.+||ou (||ç. ¹2·3), W||| |||eçu|+| |o|de|º
+ud r+||ed .+||eç+||ou o| co|o|. ||oWu, d+||
||oWu, +ud ||+c| o| |edd|º| |oueº |u| W|||ou|
ç|+, o| ||ue, +º |||º occu|º ou|, W|eu re|+·
u|u (W||||u r+c|op|+çeº) o| re|+uoc,|eº o|
re|+uor+ ce||º +|e |oc+|ed |u ||e de|r|º. I|e
c||u|c+| d|º||uc||ou |e|Weeu re|+uor+ |u º||u
+ud ºe.e|e|, +|,p|c+| d,ºp|+º||c ue.| r+, uo|
|e poºº|||e. \oº| |||e |uºu|+uce corp+u|eº +|
||e p|eºeu| ||re do uo| |eç+|d |||º |eº|ou +º +
r+||çu+uc,, |u| || de||u||e|, |º.
I|e c||u|c+| co||e|+||ouº o| \|' +|e |-¤·
|·¸¤ ¤c|·¸¤c (||ç. ¹2·1) +ud ||+| º±¤-·|·:·c|
º¤·-c!·¤¸ ¤-|c¤¤¤c (||ç. ¹2·3) +ud ||eºe +|e
d|ºcuººed |u ||e |eºpec||.e ºec||ouº |e|oW.
M£IAN0MA IN SIIü (MIS) |C|·9 . 2!2
°
|C|·¹0 . |02 
FICük£ 12-T Me|aooma ìo sìtu: |eotì¿o ma|ì¿oa A |+|çe, .e|, |||eçu|+| +ud +º,rre|||c r+cu|e ou ||e
p|e+u||cu|+| |eç|ou o| + 13·,e+|·o|d r+|e. I|e|e |º º|||||uç .+||eç+||ou o| p|çreu|+||ou (|+u, ||oWu, d+|| ||oWu,
||+c|).
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 312
I|e |e+º| corrou (<5°) o| ||e |ou| p||uc|p+|
re|+uor+ |,peº o| W|||e pe|ºouº ¦||e o||·
e|º +|e ºupe|||c|+| ºp|e+d|uç re|+uor+ (''\),
uodu|+| re|+uor+ (|\), +ud +c|+| |eu||ç|uouº
re|+uor+ (A|\)|.
|| occu|º |u o|de| pe|ºouº ou ||e roº| ºuu·
e\poºed +|e+º-||e |+ce +ud |o|e+|rº.
'uu||ç|| |º ||e roº| |rpo||+u| p+||oçeu|c |+c|o|
|u |\\.
|\\ +|W+,º º|+||º +º |-¤|·¸¤ ¤c|·¸¤c (|\), W||c|
|ep|eºeu|º + r+cu|+| |u||+ep|de|r+| ueop|+ºr
+ud |º +u \|' (||ç. ¹2·1). |\ |º ||uº uo| + p|ecu|·
ºo| |u| +u e.o|.|uç |eº|ou o| re|+uor+.
|oc+| p+pu|+| +ud uodu|+| +|e+º º|çu+| + ºW||c|
||or ||e |+d|+| |o ||e .e|||c+| ç|oW|| p|+ºe +ud
||uº |u.+º|ou |u|o ||e de|r|º, ||e |eº|ou |º uoW
c+||ed |\\ (|r+çe ¹2·¹).
|o| ||e roº| |rpo||+u| c||u|c+| c|+|+c|e||º||cº, ºee
I+||e ¹2·+.
I£NIIC0 MAIICNA M£IAN0MA (IMM) |C|·9 . 2!2
°
|C|·¹0 . |02
£FI0£MI0I0C¥
A¿e oI 0oset Median age 65.
Sex Equal incidence in males and females.
kace Raie in biown- (e.g., Asians, East
Indians) and extiemely iaie in black-skinned
(Afiican Ameiicans and Afiicans) peisons.
Highest incidence in whites and skin photo-
types I, II, and III.
Iocìdeoce 5% of piimaiy cutaneous melanomas.
Fredìsposìo¿ Factors Same factois as in sun-
induced nonmelanoma skin cancei: oldei pop-
ulation, outdooi occupations (faimeis, sailois,
constiuction woikeis).
FAIh0C£N£SIS
In contiast to SSM and NM, which appeai
to be ielated to inteimittent high-intensity
sun exposuie and occui on the inteimittently
exposed aieas (back and legs) of young oi mid-
dle-aged adults, LM and LMM occui on the
face, neck, and doisa of the foieaims oi hands
(Table 12-4); fuitheimoie, LM and LMM occui
almost always in oldei peisons with evidence
of heavily sundamaged skin (deimatoheliosis).
The evolution of the lesion most cleaily ieveals
the tiansition fiom the iadial to the veitical
giowth phase and fiom a clinically iecognizable
MIS to invasive melanoma (Image 12-1).
CIINICAI MANIF£SIAII0N
LMM veiy slowly evolves fiom LM ovei a pe-
iiod of seveial yeais, sometimes up to 20 yeais.
Theie is piactically always a backgiound of
deimatoheliosis.
Skìo Iesìoos LentIgv Mu|Ignu Unifoimly
[|aì, macule (Fig. 12-7); 0.5 cm oi laigei, up to
20 cm (Fig. 12-9A). Usually well defined, in some
aieas also bluiied boideis oi highly iiiegulai
IMAC£ 12-1 Ieotì¿o ma|ì¿oa me|aooma.
|||uº||+|ed ou ||e ||ç|| |º + |+|çe, .+||eç+|ed, ||ec||e·
|||e r+cu|e (uo| e|e.+|ed +|o.e ||e p|+ue o| ||e º||u)
W||| |||eçu|+| |o|de|º, ||e |eu +|e+º º|oW |uc|e+ºed
uur|e|º o| re|+uoc,|eº, uºu+||, +|,p|c+| +ud ||/+||e,
+ud +|e d|º||||u|ed º|uç|e |||e +|ouç ||e |+º+| |+,e|, +|
ce||+|u p|+ceº |u ||e de|r|º, r+||çu+u| re|+uoc,|eº
|+.e |u.+ded +ud |o|red p|or|ueu| ueº|º. A| ||e
|e|| |º + |+|çe uodu|e ||+| |º corpoºed o| ep|||e||o|d
ce||º |u |||º |||uº||+||ou, ||e uodu|eº o| +|| |ou| r+|u
ºu||,peº o| re|+uor+ +|e |ud|º||uçu|º|+||e ||or
e+c| o||e|.
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 313
â 8
FICük£ 12-8 Me|aooma ìo sìtu, superIìcìa| spreadìo¿ type â. B+|e|, e|e.+|ed p|+que ou ||e +|r o| +
15·,e+|·o|d W|||e r+|e W+º |||º| uo|ed 5 ,e+|º p|e.|ouº|,, ç|+du+||, |uc|e+º|uç |u º|/e. I|e |eº|ou |º +º,rre|||c
+ud ||e|e |º +|ºo +º,rre||, |u ||e d|º||||u||ou o| co|o| ||+| |º .+||eç+|ed +ud º|oWº d+||·||oWu ºpec|º +ç+|uº| +
|+u |+c|ç|ouud. |e|r+|op+||o|oç, o| ||e |eº|ou º|oWed + ºupe|||c|+| ºp|e+d|uç re|+uor+ |u º||u. 8. Au +|roº|
o.+|, |+|e|, e|e.+|ed ºr+|| p|+que ||+| |+º + |e|+||.e|, |eçu|+| |o|de| |u| |º º|||||uç W||| |eç+|d |o ||e .+||eç+||ou
|u co|o|. |+u, d+|| ||oWu, +ud e.eu ||+c| W||| +u o|+uçe po|||ou ou ||e ||ç||. |e|r+|op+||o|oç, +ç+|u º|oWed
\|' W||| + p+çe|o|d ç|oW|| p+||e|u o| |u||+ep|de|r+| re|+uor+ ce||º.
FICük£ 12-9 Ieotì¿o ma|ì¿oa â. A .e|, |+|çe |eu||ço r+||çu+ ou ||e ||ç|| c|ee| W||| ||e |,p|c+| .+||eç+·
||ou |u co|o| (|+u, ||oWu, ||+c|) +ud ||ç||, |||eçu|+| º|+pe. I|e |eº|ou |º ||+|, r+cu|+|, +ud ||uº |ep|eºeu|º |u º||u
re|+uor+. 8. I|e c|+ºº|c+||, r+cu|+| |eu||ço r+||çu+ |º ||ç||, |||eçu|+| |u º|+pe +ud .+||eç+|ed |u co|o|. noW·
e.e|, ||e|e |º + ||u|º| corpoueu| +ud + |+|çe p|u| uodu|e |u ||e |u||+o||||+| |eç|ou, |ud|c+||uç + ºW||c| ||or ||e
|+d|+| |o ||e .e|||c+| ç|oW|| p|+ºe +ud ||uº |u.+º|.eueºº. ||e |eº|ou |º uoW c+||ed |eu||ço r+||çu+ re|+uor+.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 314
boideis, often with a notch; °geogiaphic" shape
with inlets and peninsulas (Fig. 12-9B). Eaily
lesions tan, advanced lesions: stiiking vaiiations
in hues of biown and black (speckled), appeais
like a °stain" (Fig. 12-7); haphazaid netwoik of
black on a backgiound of biown (Fig. 12-9À).
No hues of ied and blue.
LentIgv Mu|Ignu Me|unvmu The clinical
change that indicates the tiansition of LM to
LMM is the appeaiance of vaiiegated ied, white,
and blue and of papules, plaques, oi nodules
(see Fig. 12-9B). Thus LMM is the same as LM
¡|us (1) giay aieas (indicate focal iegiession),
and blue aieas ¦indicating deimal pigment
(melanocytes oi melanin)], and (2) papules oi
nodules, which may be blue, black, oi pink (Fig.
12-9B). Raiely, LMM may be nonpigmented. It
is then skin-coloied and patchy ied and clini-
cally not diagnosable (see Fig. 12-15À).
DIstrIhutIvn Single isolated lesion on the sun-
exposed aieas: foiehead, nose, cheeks, neck, foie-
aims, and doisa of hands; iaiely on lowei legs.
0ther Skìo Chao¿es ìo Areas oI Iumor Sun-
induced changes: solai keiatosis, fieckling,
telangiectasia, thinning of the skin, i.e., deima-
toheliosis.
Ceoera| Medìca| £xamìoatìoo Check foi ie-
gional lymphadenopathy.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y LM shows incieased num-
beis of atypical melanocytes distiibuted in a
single layei along the basal layei and above
the basement membiane of an epideimis
that shows elongation of iete iidges. Atypi-
cal melanocytes aie usually singly dispeised
but may also aggiegate to small nests and
extend into the haii follicles, ieaching the mid-
deimis, even in the pieinvasive stage of LM.
In LMM, they invade the deimis (veitical
giowth phase) and expand into the deepei tis-
sues (Image 12-1).
IA8I£ 12-5 Cutaneous Ne|anoma: Sta¿e Croupin¿ and Pro¿nosis
Stage 0||o|ca| Stag|og Patho|og|c Stag|og S0rv|va|,%
I | \ I | \
0 I|º |0 \0 I|º |0 \0
|A I¹+ |0 \0 I¹+ |0 \0 95
|B I¹| |0 \0 I¹| |0 \0 90
I2+ |0 \0 I2+ |0 \0
||A I2| |0 \0 I2| |0 \0 13
I!+ |0 \0 I!+ |0 \0
||B I!| |0 \0 I!| |0 \0 o5
I++ |0 \0 I++ |0 \0
||C I+| |0 \0 I+| |0 \0 +5
||| Au, I |¹ \0
|||A I¹-++ |¹+ \0 o0
I¹-++ |2+ \0
|||B I¹-+| |¹+ \0
I¹-+| |2+ \0
I¹-++ |¹| \0 52
I¹-++ |2| \0
I¹-++/| |2c \0
|||C I¹-+| |¹| \0
I¹-+| |2| \0 2o
Au, I |! \0
|\ Au, I Au, | Au, \¹ Au, I Au, | Au, \¹ 1.5-¹¹
'ou|ce. Ad+p|ed ||or C\ B+|c| e| +|. l C||u 0uco| ¹9.!o22-!+, 200¹.
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 315
0IFF£k£NIIAI 0IACN0SIS
varìe¿ate Iao-8rowo Macu|e[Fapu|e[Nodu|e
Se|orr|eít |eraìoses may be daik but aie exclu-
sively papules oi plaques and have a chaiac-
teiistic stippled suiface, often with a veiiucous
component, i.e., a °waity" but gieasy suiface that,
when sciatched, exhibits fine scales . So|ar |enìígo ,
although maculai, does not exhibit the intensity
oi vaiiegation of biown, daik biown, and black
hues seen in LM. Deimoscopy is essential.
Fk0CN0SIS
Summaiized in Tables 12-5 and 12-6.
MANAC£M£NI
See also page 332.
1. Veiy eaily LM lesions: Imiquimod.
2. Excise with 1-cm beyond the clinically vis-
ible lesion wheie possible and piovided the
'upe|||c|+| ºp|e+d|uç re|+uor+ (''\) |º ||e
roº| corrou re|+uor+ (10°) |,pe |u pe|ºouº
W||| W|||e º||u.
|| +||ºeº roº| ||equeu||, ou ||e uppe| |+c| +ud
occu|º +º + rode|+|e|, º|oW·ç|oW|uç |eº|ou o.e|
+ pe||od up |o 2 ,e+|º.
''\ |+º + d|º||uc||.e ro|p|o|oç,. +u e|e.+|ed,
||+| |eº|ou (p|+que). I|e p|çreu| .+||eç+||ou o|
''\ |º º|r||+| |o, |u| ro|e º|||||uç ||+u, ||e
.+||e|, o| co|o| p|eºeu| |u roº| |\\. I|e co|o|
d|ºp|+, |º + r|\|u|e o| ||oWu, d+|| ||oWu, ||+c|,
||ue, +ud |ed, W||| º|+|e·ç|+, o| ç|+, |eç|ouº |u
+|e+º o| |uro| |eç|eºº|ou.
|o| roº| |rpo||+u| c||u|c+| c|+|+c|e||º||cº, ºee
I+||e ¹2·+.
SüF£kFICIAI SFk£A0INC M£IAN0MA |C|·9 . 2!2
°
|C|·¹0 . |02
£FI0£MI0I0C¥
A¿e oI 0oset 30 to 50 (median, 37) yeais of
age.
Sex Slightly highei incidence in females.
kace In woild suiveys, white-skinned pei-
sons oveiwhelmingly piedominate. Only 2%
biown- oi black-skinned. Fuitheimoie, biown
and black peisons have melanomas usually oc-
cuiiing on the extiemities; half of biown and
black peisons have piimaiy melanomas aiising
on the sole of the foot (see below).
Iocìdeoce SSM constitutes 70% of all melano-
mas aiising in white peisons.
Fredìsposìo¿ aod kìsk Factors (see Iab|e 12-3)
In oidei of impoitance these aie ¡resente o[ ¡re-
tursor |esíons (DN, CNMN; pages 300 and 304);
[amí|y |ísìory of melanoma in paients, childien,
oi siblings; |íg|ì s|ín to|or (skin phototypes
I and II); and sunbuins, especially duiing pieado-
lescence. Especially incieased incidence in young
uiban piofessionals, with a fiequent pattein of
inteimittent, intense sun exposuie (°weekend-
eis") oi wintei holidays neai the equatoi.
FAIh0C£N£SIS
In the eaily stages of giowth theie is an in-
tiaepideimal, oi iadial, giowth phase duiing
which tumoiigenic pigment cells aie confined
to the epideimis and thus cannot metastasize.
At this stage SSM is an MIS (Fig. 12-8 and
Image 12-2). This °giace peiiod" of the iadial
giowth phase, with potential foi cuie, is fol-
lowed by the invasive veitical giowth phase, in
which malignant cells consist of a tumoiigenic
nodule that veitically invades the deimis with
potential foi metastasis (Image 12-2).
IA8I£ 12-6 8·Year Surviva| Rates for
Patients with C|inica| Sta¿e | Ne|anoma (|n
the Vertica| Crowth Phase) Based on Iumor
Ihickness
Th|ckoess, mm 8-Year S0rv|va| 8ate, %
<0.1o 9!.2
0.1o-¹.o9 35.o
¹.10-!.o0 59.3
>!.o0 !!.!
'ou|ce. Ad+p|ed ||or wn C|+|| l| e| +|. l |+|| C+uce| |uº| 3¹.¹39!,
¹939.
flat component does not involve a majoi
oigan. Use of Wood lamp and deimoscopy
help in defining boideis.
3. Sentinel node to be done in lesions >1.0 mm
in teims of thickness.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 316
The pathophysiology of SSM is not yet undei-
stood. Ceitainly, in some consideiable numbei
of SSMs, sunlight exposuie is a factoi, and SSM
is ielated to occasional buists of iecieational
sun exposuie duiing a susceptible peiiod (<14
yeais). About 10% of the SSMs occui in high-
iisk families. The iest of the cases may occui
spoiadically among peisons without a specific
genetic iisk.
CIINICAI MANIF£SIAII0N
The usual histoiy of SSM is a change in a pie-
viously existing pigmented lesion (mostly a
DN). It should be noted, howevei, that 70% of
melanomas aiise in °noimal" skin, but since ini-
tial giowth is slow and melanomas often occui
in peisons with many nevi, an eaily SSM may be
mistaken foi a pieexisting nevus by the patient.
Often, a patient may offei a histoiy of having
had a mole at that paiticulai site since child-
hood (°as long as I can iemembei"), but when
a photogiaph of that paiticulai age peiiod and
site is ietiieved fiom a family album, no such
°mole" can be detected.
The patient oi a close ielative may note a
giadual daikening in one aiea of a °mole" (see
Figs. 12-3, 12-8) oi a change in shape; and
as the daik aieas inciease theie will develop
vaiiegation of coloi with mixes of biown, daik
biown, and black. Also, the boideis of a pievi-
ously iegulaily shaped lesion may become ii-
iegulai with pseudopods and a notch.
With the switch fiom the iadial to a veitical
giowth phase (Image 12-2), and thus invasion
into the deimis, theie is the clinical appeai-
ance of a papule and latei nodule on top of
the slightly elevated plaque of an SSM. Since
many SSMs initially have the potential foi a
tumoi-infiltiating lymphocyte (TIL)-mediated
iegiession, albeit only paitial, othei aieas of the
SSM plaque may sink to the level of suiiound-
ing noimal skin and the coloi mixes of biown
to black aie expanded by the addition of ied,
white, and the tell-tale blue and blue-giay.
Skìo Iesìoos (Figs. 12-10 and 12-11)
SSM is the lesion to which the ABCDE iule
(page 310) best applies. Initially a veiy flat
plaque 5-12 mm oi smallei (Fig. 12-8); oldei
lesions, 10-25 mm (Fig. 12-10). Asymmetiic
(one half unlike the othei) (Figs. 12-10À, B,
and C) oi oval with iiiegulai boideis (Fig. 12-
10D) and often with one oi moie indentations
(notches) (Figs. 12-10 and 12-11). Shaiply
defined. Daik biown, black, with admixtuie of
pink, giay, and blue-giay hues-with maiked
vaiiegation and a haphazaid pattein. White
aieas indicate iegiessed poitions (Figs. 12-10C
IMAC£ 12-2 SuperIìcìa| spreadìo¿
me|aooma I|e |o|de| |º |||eçu|+| +ud
e|e.+|ed |||ouç|ou| ||º eu|||e|,, ||opº, o|
|||º p|+que ºu||ouud|uç ||e |+|çe uodu|e
º|oWº + p+çe|o|d d|º||||u||ou o| |+|çe
re|+uoc,|eº |||ouç|ou| ||e ep|de|r|º |u
ru|||p|e |+,e|º, occu|||uç º|uç|, o| |u ueº|º,
+ud uu||o|r|, +|,p|c+|. 0u ||e |e|| |º + |+|çe
uodu|e, +ud ºc+||e|ed |||ouç|ou| ||e ºu|·
|ouud|uç po|||ou o| ||e p|+que +|e ºr+||e|
p+pu|+| +ud uodu|+| +|e+º. I|e uodu|eº r+,
+|ºo º|oW ep|||e||o|d, ºp|ud|e ce||º o| ºr+||
r+||çu+u| re|+uoc,|eº +º |u |eu||ço r+||çu+
re|+uor+ +ud uodu|+| re|+uor+.
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 31T
and D). An SSM is thus a flat plaque with all
shades of biown to black plus the Ameiican
flag oi the tiicoloie (ied, blue, white) (Fig.
12-10D). No |enígn ¡ígmenìeJ |esíon |as ì|ese
t|aratìerísìíts. As the veitical giowth phase
piogiesses, nodules appeai; eventually eiosions
and even supeificial ulceiation develop (Figs.
12-11C and D).
DIstrIhutIvn Isolated, single lesions; multiple
piimaiies aie iaie. Back (males and females);
legs (females, between knees and ankles); an-
teiioi tiunk and legs in males; ielatively fewei
lesions on coveied aieas, e.g., buttocks, lowei
abdomen, bia aiea.
0ermoscopy Incieases diagnostic accuiacy by
ovei 50%.
Ceoera| £xamìoatìoo Always seaich foi en-
laiged iegional nodes.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Malignant melanocytes
expand in a pagetoid pattein, i.e., in multiple
layeis within the epideimis (if confined to the
epideimis, the lesion is an MIS) and supeifi-
cial papillaiy body of the deimis-the iadial
giowth phase. They occui singly and in nests
(see Image 12-2) and aie S-100 and HMB-45 posi-
tive. In the veitical giowth phase, piesenting clini-
cally as small nodules, they expand fuithei into
the ieticulai deimis and beyond (Image 12-2). Foi
miciostaging see Table 12-7 and p. 331.
FICük£ 12-10 SuperIìcìa| spreadìo¿ me|aooma, radìa| ¿rowth phase â. A ||+|·|opped, e|e.+|ed,
+º,rre|||c +ud |||eçu|+| p|+que W||| .+||eç+|ed co|o| (||oWu, ||+c|) ou ||e ||uu| W||| º|+|p|, der+|c+|ed
r+|ç|uº. I|e ºu||+ce |º +|ºo |||eçu|+| W||| + co|||eº|oue p+||e|u. 8. Au +º,rre|||c, ||+| p|+que W||| |||eçu|+| +ud
º|+|p|, de||ued r+|ç|uº +ud + co|||eº|oue·|||e ºu||+ce. I|e re|+u|u p|çreu|+||ou |+uçeº ||or ||ç|| ||oWu |o
d+|| ||oWu, ||+c|, +ud ||e|e +|e ||ç||e| +|e+º |u|e|ºpe|ºed. C. A ||ç||, |||eçu|+| |eº|ou W||| d+||·||oWu |o ||u·
|º|·||+c| p+pu|eº |o|r|uç + ||uç +|ouud + W|||e r+cu|+| +|e+ W||| + ceu||+| ||oWu|º| |o ||u|º| p+pu|e. I||º W|||e
+|e+ r+||º ºpou|+ueouº |eç|eºº|ou. 0. A |e|+||.e|, º,rre|||c |u| |+|çe (3 cr) p|+que W||| º|+|p|, de||ued +ud
uo|c|ed |o|de| +ud + couº|de|+||e .+||eç+||ou o| co|o|. ||+c|, ||ue, |ed, +ud W|||e.
â
C
8
0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 318
C0ükS£ AN0 Fk0CN0SIS
Left untieated, SSM develops deep invasion
(veitical giowth) ovei months to yeais. Piogno-
sis is summaiized in Tables 12-5 and 12-6.
0IACN0SIS
Clinically accoiding to the ABCDE iule, veii-
fied by deimoscopy. In case of doubt, |ío¡sy ;
total excisional biopsy with naiiow maigins is
optimal biopsy pioceduie. Incisional oi punch
biopsy acceptable when total excisional biopsy
cannot be peifoimed oi when lesion is laige,
iequiiing extensive suigeiy to iemove the entiie
lesion.
MANAC£M£NI
Sur¿ìca| Ireatmeot See page 332.
IA8I£ 12-T Ne|anoma INN C|assification
T 0|ass|I|cat|oo Th|ckoess, mm 0|cerat|oo Stat0s
I ¹ ¹.0 +. w|||ou| u|ce|+||ou +ud |e.e| ||/|||
c
|. w||| u|ce|+||ou o| |e.e| |\/\/I2
c
I2 ¹.0¹-2.0 +. w|||ou| u|ce|+||ou
|. w||| u|ce|+||ou
I! 2.0¹-+.0 +. w|||ou| u|ce|+||ou
|. w||| u|ce|+||ou
I+ >+.0 +. w|||ou| u|ce|+||ou
|. w||| u|ce|+||ou
h 0|ass|I|cat|oo ho. oI Netastat|c hodes hoda| Netastat|c Nass
|¹ ¹ +. \|c|ore|+º|+º|º
|. \+c|ore|+º|+º|º
|2 2-! +. \|c|ore|+º|+º|º
|. \+c|ore|+º|+º|º
c. |u·||+uº|| re|(º)/º+|e||||e(º)
W|||ou| re|+º|+||c uodeº
|! + o| ro|e re|+º|+||c
uodeº, o| r+||ed
uodeº, o| |u·||+uº||
re|(º)/º+|e||||e(º)
W||| re|+º|+||c
uode(º)
N 0|ass|I|cat|oo S|te Ser0m Lactate 0ehydrogeoase
\¹+ ||º|+u| º||u, |o|r+|
ºu|cu|+ueouº,
o| uod+| re|+º|+ºeº
\¹| |uuç re|+º|+ºeº |o|r+|
\¹c A|| o||e| .|ºce|+| re|+º|+ºeº |o|r+|
Au, d|º|+u| E|e.+|ed
re|+º|+º|º
c
C|+|| |e.e| |, |u||+ep|de|r+|, |e.e| ||, |u.+deº p+p|||+|, de|r|º, |e.e| |||, ||||º p+p|||+|, de|r|º, |e.e| |\, |u.+deº |e||cu|+| de|r|º, |e.e| \, |u.+deº
ºu|cu|+ueouº |+|.
'ou|ce. Ad+p|ed ||or C\ B+|c| e| +|. l C||u 0uco| ¹9.!o!5, 200¹.
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 319
FICük£ 12-11 SuperIìcìa| spreadìo¿ me|aooma, vertìca| ¿rowth phase â. Au ou|, r|u|r+||, |||eçu|+|
p|+que W||| .+||eç+|e co|o| (||oWu, ||+c|). |u ||e ceu|e| ||e|e |º + ºr+|| ||+c|, dore·º|+ped uodu|e. I||º |º ||e
ºW||c| |o ||e .e|||c+| ç|oW|| p|+ºe. 8. Au |||eçu|+| .e|, ||+| p|+que W||| uo|c|ed |o|de|º +ud ||ç||, .+||eç+|ed
co|o| (|+u, ||oWu, ||+c|, +ud |ed). '||ç|||, o|| ceu|e| ||e|e |º + |+|çe p+|||+||, c|uº|ed uodu|e (.e|||c+| ç|oW||
p|+ºe). C. A ||ç||, |||eçu|+| +ud +º,rre|||c p|+que W||| + co|||eº|oue·|||e ºu||+ce +ud .+||eç+|ed co|o| (||+c|,
||oWu). 0u ||e ||ç|| ||e|e |º +u e\ceu|||c e|oded ||+c| |o ||ue uodu|e |ep|eºeu||uç ||e .e|||c+| ç|oW|| p|+ºe.
0. A ||ç||, |||eçu|+|, +º,rre|||c ||u|º| |o ||+c| p|+que W||| ||oWu, |ed, +ud W|||e (|eç|eºº|ou). 0|| ceu|e| |º +u
e|oded ||+c| uodu|e (.e|||c+| ç|oW||).
â
C
8
0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 320
|odu|+| re|+uor+ (|\) |º ºecoud |u ||equeuc,
+||e| ''\.
0ccu|||uç |+|çe|, |u r|dd|e |||e |u pe|ºouº W|||
W|||e º||u +ud, +º |u ''\, ou ||e |eºº corrou|,
e\poºed +|e+º.
I|e |uro| ||or ||e |eç|uu|uç |º |u ||e .e|||c+|
ç|oW|| p|+ºe (|r+çe ¹2·!).
|\ |º uu||o|r|, e|e.+|ed +ud p|eºeu|º +º + |||c|
p|+que o| +u e\op|,||c, po|,po|d o| dore·º|+ped
|eº|ou.
I|e co|o| p+||e|u |º uºu+||, uo| .+||eç+|ed, +ud
||e |eº|ou |º uu||o|r|, ||ue o| ||ue·||+c| o|,
|eºº corrou|,, c+u |e .e|, ||ç|||, p|çreu|ed o|
uoup|çreu|ed (+re|+uo||c re|+uor+).
|\ |º ||e oue |,pe o| p||r+|, re|+uor+ ||+|
+||ºeº qu||e |+p|d|, (¹2 rou||º |o 2 ,e+|º) ||or
uo|r+| º||u o| ||or + re|+uoc,||c ue.uº +º +
uodu|+| (.e|||c+|) ç|oW|| W|||ou| +u +dj+ceu| ep|·
de|r+| corpoueu|, +º |º +|W+,º p|eºeu| |u |\\
+ud ''\ (ºee |r+çeº ¹2·¹ +ud ¹2·2).
|o| ||e roº| |rpo||+u| c||u|c+| c|+|+c|e||º||cº, ºee
I+||e ¹2·+.
N00üIAk M£IAN0MA |C|·9 . 2!2
°
|C|·¹0 . |02
IMAC£ 12-3 Nodu|ar me|aooma I||º +||ºeº +| ||e de|r+|·ep|de|r+| juuc||ou +ud e\|eudº .e|||c+||, |u ||e
de|r|º. I|e ep|de|r|º |+|e|+| |o ||e +|e+º o| |||º |u.+º|ou doeº uo| derouº||+|e +|,p|c+| re|+uoc,|eº. Aº |u |eu·
||ço r+||çu+ re|+uor+ +ud ºupe|||c|+| ºp|e+d|uç re|+uor+, ||e |uro| r+, º|oW |+|çe ep|||e||o|d ce||º, ºp|ud|e
ce||º, ºr+|| r+||çu+u| re|+uoc,|eº, o| r|\|u|eº o| +|| |||ee
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 321
£FI0£MI0I0C¥
A¿e oI 0oset Middle life.
Sex Equal incidence in males and females.
kace NM occuis in all iaces, but in the Japa-
nese it occuis eight times moie fiequently
(27%) than SSM (3%).
Iocìdeoce NM constitutes 15% (up to 30%) of
the melanomas in the United States.
Fredìsposìo¿ aod kìsk Factors See page 309
and Table 12-3.
FAIh0C£N£SIS
Both SSM and NM occui in appioximately the
same sites (uppei back in males, lowei legs in fe-
males), and piesumably the same pathogenetic
factois aie opeiating in NM as weie desciibed
in SSM. Foi the giowth pattein of NM, see Im-
age 12-3. The ieason foi the high fiequency of
NM in the Japanese is not known.
CIINICAI MANIF£SIAII0N
This type of melanoma may aiise in a pieexist-
ing nevus, but moie commonly aiises de novo
fiom noimal skin. In contiast to SSM, NM
evolves ovei a few months and is often noted
by the patient as a new °mole" that was not
piesent befoie.
Skìo Iesìoos Unifoimly elevated °bluebeiiy-
like" nodule (Figs. 12-12À and B) oi ulceiated
oi °thick" plaque; may become polypoid.
Unifoimly daik blue, black, oi °thundeicloud"
giay (Fig. 12-12À, B); lesions may appeai pink
with a tiace of biown oi a black iim (amelanotic
NM, see Fig. 12-15C). Suiface smooth oi scaly,
eioded (Fig. 12-12C) oi ulceiated (Fig. 12-
12D). Eaily lesions aie 1-3 cm in size but
may giow much laigei if undetected. Oval
oi iound, usually with smooth, not iiiegulai,
boideis, as in all othei types of melanoma.
Shaiply defined, may be pedunculated (Fig.
12-12D).
DIstrIhutIvn Same as SSM. In the Japanese,
NM occuis on the extiemities (aims and legs).
Ceoera| Medìca| £xamìoatìoo Always seaich
foi nodes.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Malignant melanocytes,
which appeai as epithelioid, spindle, oi small
atypical cells, show little lateial (iadial) giowth
within and below the epideimis and invade
veitically into the deimis and undeilying sub-
cutaneous fat (see Image 12-3). They aie S-100
and usually HMB-45 positive. Foi miciostag-
ing, see page 331.
Sero|o¿y Seium levels of S-100 beta
and melanoma-inhibiting activity (MIA),
S-cysteinyldopa, and lactate dehydioge-
nase (LDH) levels aie maikeis foi aJ·anteJ
melanoma patients. LDH is to date the only
statistically significant maikei foi ¡rogressí·e
disease.
0IACN0SIS
Clinical and with the help of deimoscopy. How-
evei, deimoscopy may fail in unifoimly black
lesions. In case of doubt, |ío¡sy . Total excisional
biopsy with naiiow maigins is optimal biopsy
pioceduie, wheie possible. If biopsy is positive
foi melanoma, ieexcision of site will be neces-
saiy (see Management, p. 332). Incisional oi
punch biopsy acceptable when total excisional
biopsy cannot be peifoimed oi when lesion is
laige, iequiiing extensive suigeiy to iemove the
entiie lesion.
0IFF£k£NIIAI 0IACN0SIS
8|ue[8|ack Fapu|e[Nodu|e NM can be con-
fused with |emangíoma (long histoiy) and
¡yogenít granu|oma (shoit histoiy-weeks) (see
Fig. 12-12C) and is sometimes almost indis-
tinguishable fiom ¡ígmenìeJ |asa| te|| tartí-
noma , although it is usually softei. Howevei,
any °bluebeiiy-like" nodule of iecent oiigin
(6 months to 1 yeai) should be excised oi, if
laige, an incisional biopsy is mandatoiy foi
histologic diagnosis.
Fk0CN0SIS
Summaiized in Tables 12-5 and 12-6.
MANAC£M£NI
Sur¿ìca| Ireatmeot See page 332.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 322
FICük£ 12-12 Nodu|ar me|aooma â. A 9·rr dore·º|+ped ºroo|| uodu|e W||| + ||+||e| ||oWu|º| ||r
+||º|uç ou ||e |+c| o| + !3·,e+|·o|d r+|e. 8. A ¹·cr ||+c| p+pu|e ou ||e poº|e||o| |||ç| o| + o0·,e+|·o|d |er+|e.
I|e |eº|ou |+d |eeu p|eºeu| |o| |eºº ||+u ¹ ,e+|. C. Au e|oded, ||eed|uç, ||oWu uodu|e |+.|uç + ruº||oor·|||e
cou||çu|+||ou ç|.|uç || + º|uc|·ou +ppe+|+uce. 'uc| |eº|ouº c+u |e r|º|+|eu |o| + .+ºcu|+| |eº|ou ºuc| +º + p,o·
çeu|c ç|+uu|or+. 0. |+|çe (5 cr) |||eçu|+|, ||+c|, ||eed|uç uodu|e º||||uç ou ||e º||u |||e + ruº||oor. I|e |eº|ou
|+d ç|oWu |o| o.e| + |+|| ,e+| +ud ||e 5o·,e+|·o|d r+|e p+||eu| |+d uo| ºeeu + p|,º|c|+u ou| o| |e+| '|| r|ç|| |e
re|+uor+.¨
â
C
8
0
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 323
I|e |e|r !-º¤¤¤|cº·c |e|e|º |o couuec||.e ||ººue
p|o|||e|+||ou +ud, W|eu +pp||ed |o r+||çu+u|
re|+uor+, deºc|||eº (¹) + de|r+| ||||o||+º||c
corpoueu| o| re|+uor+ W||| ou|, r|u|r+|
re|+uoc,||c p|o|||e|+||ou +| ||e de|r+|·ep|de|r+|
juuc||ou, (2) ue|.e·ceu|e|ed ºupe|||c|+| r+||çu+u|
re|+uor+ W||| o| W|||ou| +u +|,p|c+| |u||+ep|de|·
r+| re|+uoc,||c corpoueu|, o| (!) o||e| |eº|ouº
|u W||c| ||e |uro| +ppe+|º |o +||ºe |u |eu||ço
r+||çu+ o|, |+|e|,, |u +c|+| |eu||ç|uouº re|+uor+
o| ºupe|||c|+| ºp|e+d|uç re|+uor+.
A|ºo, |\ ç|oW|| p+||e|uº |+.e |eeu uo|ed |u
|ecu||eu| r+||çu+u| re|+uor+.
|\ r+, |e + .+||+u| o| |\\ |u ||+| roº| |eº|ouº
occu| ou ||e |e+d +ud uec| |u p+||eu|º W||| de|·
r+|o|e||oº|º.
|\ |º ro|e |||e|, |o |ecu| |oc+||, +ud re|+º|+º|/e
||+u |\\, |oWe.e|. |\ |º |+|e +ud occu|º ro|e
||equeu||, |u Woreu +ud pe|ºouº >55 ,e+|º o|d.
A| d|+çuoº|º |\ |eº|ouº |+.e |eeu p|eºeu| ||or
rou||º |o ,e+|º. |\ |º +º,rp|or+||c, uºu+||, uo|
p|çreu|ed +ud |º ||e|e|o|e o.e||oo|ed |, ||e
p+||eu|. E+||, |eº|ouº r+, +ppe+| +º .+||eç+|ed
|eu||ç|uouº r+cu|eº o| p|+queº, +| ||reº W|||
ºr+|| ||ue·ç|+, ºpec|º o| co|o| (||ç. ¹2·¹!1).
|+|e| |eº|ouº r+, +ppe+| +º de|r+| uodu|eº,
+ud +|||ouç| ||e, corrou|, |+c| +u, re|+u|u
p|çreu|+||ou, ||e, r+, |+.e ç|+, |o ||ue p+pu|+|
e|e.+||ouº (||ç. ¹2·¹!3). Bo|de|º, W|eu d|ºce|u·
|||e, +|e |||eçu|+| +º |u |\.
I|e d|+çuoº|º |equ||eº +u e\pe||euced de|r+|·
op+||o|oç|º|, '·¹00 |rruuope|o\|d+ºe·poº|||.e
ºp|ud|e ce||º ueed |o |e |deu||||ed |u ||e r+|||\
co||+çeu. n\B·+5 º|+|u|uç r+, |e ueç+||.e. A
|,p|c+| juuc||ou+| re|+uoc,||c p|o|||e|+||ou, e|||e|
|ud|.|du+| o| |oc+| ueº|º, occu|º, |eºer|||uç |\.
'·¹00·poº|||.e ºp|ud|e·º|+ped ce||º +|e er|edded
|u r+|||\ co||+çeu ||+| W|de|, ºep+|+|eº ||e ºp|u·
d|e ce|| uuc|e|. 'r+|| +çç|eç+|eº o| |,rp|oc,|eº
+|e corrou|, ºeeu +| ||e pe||p|e|, o| |\. |eu·
|o||op|ºr |º c|+|+c|e||º||c, |.e., ||||o||+º|·|||e |u·
ro| ce||º +|ouud o| W||||u eudoueu||ur o| ºr+||
ue|.eº. 0||eu, |\ |º ºeeu W||| + |+c|ç|ouud o|
ºe.e|e ºo|+| d+r+çe |o ||e de|r|º.
I|e|e +|e r|\ed .|eWº +|ou| ||e p|oçuoº|º o|
|\. |u oue ºe||eº, +pp|o\|r+|e|, 50° o| p+||eu|º
e\pe||euced + |oc+| |ecu||euce +||e| p||r+|,
e\c|º|ou o| |\, uºu+||, W||||u ! ,e+|º o| e\c|º|ou,
ºore p+||eu|º e\pe||euced ru|||p|e |ecu||euceº.
|,rp| uode re|+º|+º|º occu|º |eºº o||eu ||+u
|oc+| |ecu||euce. |u oue ºe||eº, 20° de.e|oped
re|+º|+ºeº, +ud |\ W+º |eç+|ded +º + ro|e +ç·
ç|eºº|.e |uro| ||+u |\\.
|o| r+u+çereu| ºee p+çe !!2.
0£SM0FIASIIC M£IAN0MA (0M)
FICük£ 12-13 0esmop|astìc me|aooma â. Are|+uo||c deºrop|+º||c re|+uor+. A ||+|, º||u·co|o|ed uod·
u|e W||| + ºpec| o| ||oWu |u ||e ceu|e| ||+| +ppe+|ed ou ||e |o|e|e+d o| |||º +3·,e+|·o|d |er+|e. 8. A ||+| uodu|e
W||| ||u|º|·|ed +ud ||oWu po|||ou |u +u e|de||, r+|e, |eº|ouº o||eu +|e ºu||ouuded |, + r+cu|+| po|||ou |eºer·
|||uç |eu||ço r+||çu+.
â 8
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 324
£FI0£MI0I0C¥
A¿e oI 0oset Median age is 65.
Iocìdeoce 7 to 9% of all melanomas; in whites,
2 to 8%.
Sex Male:female iatio, 3:1.
kace ALM is the piincipal melanoma in the
Japanese (50-70%) and in Ameiican and sub-
Sahaian Afiican blacks.
FAIh0C£N£SIS
The pigmented macules that aie fiequently
seen on the soles of Afiican blacks could be
compaiable with DN. ALM has a similai giowth
pattein as LMM.
CIINICAI MANIF£SIAII0N
ALM is slow giowing (about 2.5 yeais fiom
appeaiance to diagnosis). The tumois occui
on the volai suiface (palm oi sole) and in theii
iadial giowth phase may appeai as a giadually
enlaiging °stain." ALM as subungual (thumb oi
gieat toe) melanoma appeais fiist in the nail
bed and involves, ovei a peiiod of 1 to 2 yeais,
the nail matiix, eponychium, and nail plate. In
the veitical giowth phase nodules appeai; often
theie aie aieas of ulceiation, and nail defoimity
and shedding of the nail may occui.
Skìo Iesìoos Acra| aod Fa|m[So|e Maculai
oi slightly iaised lesion in the iadial giowth
phase (Fig. 12-14), with focal papules and
nodules developing duiing the veitical giowth
phase. Maiked vaiiegation of coloi including
biown, black, blue, depigmented pale aieas (Fig.
12-14). Iiiegulai boideis as in LMM; usually
well defined but not infiequently ill defined.
This type of ALM occuis on soles, palms, doisal
and palmai/plantai aspects of fingeis and toes
(Figs. 12-14C and D).
Subuo¿ua| Subungual macule beginning at
the nail matiix and extending to involve the
Ac|+| |eu||ç|uouº re|+uor+ (A|\) |º + ºpec|+|
p|eºeu|+||ou o| cu|+ueouº re|+uor+ +||º|uç ou
||e ºo|e, p+|r, +ud ||uçe|u+|| o| |oeu+|| |ed.
A|\ occu|º roº| o||eu |u Aº|+uº, ºu|·'+|+|+u
A|||c+uº, +ud A|||c+u Are||c+uº, corp||º|uç
50-10° o| ||e re|+uor+º o| ||e º||u |ouud |u
||eºe popu|+||ouº.
|| occu|º roº| o||eu |u o|de| r+|eº ( o0 ,e+|º)
+ud o||eu ç|oWº º|oW|, o.e| + pe||od o| ,e+|º.
I|e de|+, |u de.e|opreu| o| ||e |uro| |º ||e |e+·
ºou ||eºe |uro|º +|e o||eu d|ºco.e|ed ou|, W|eu
uodu|eº +ppe+| o|, |u ||e c+ºe o| u+|| |u.o|.e·
reu|, ||e u+|| |º º|ed, ||e|e|o|e, ||e p|oçuoº|º |º
poo|.
ACkAI I£NIICIN0üS M£IAN0MA |C|·9 . 2!2
°
|C|·¹0 . |02
nail bed and nail plate. Papules, nodules, and
destiuction of the nail plate may occui in the
veitical giowth phase (Figs. 12-14B, 33-14).
Daik biown oi black pigmentation that may
involve the entiie nail and suiiounding skin
looking like LM (Figs. 12-14À and B). As the
lesion switches to the veitical giowth phase, a
papule oi nodule appeais and the nail is shed
(Figs. 12-14À and B). Often the nodules oi
papules aie unpigmented. Amelanotic ALM is
often oveilooked foi months and, since theie
aie no pigmentaiy changes, may fiist piesent as
nail dystiophy.
0IFF£k£NIIAI 0IACN0SIS
ALM (plantai type) is not infiequently iegaided
as a °plantai wait" and tieated as such. Deimos-
copy is of decisive help. Also, often misdiag-
nosed as tinea nigia.
Subuo¿ua| 0ìsco|oratìoo ALM (subungual) is
usually consideied to be tiaumatic bleeding
undei the nail, and subungual hematomas may
peisist foi ovei 1 yeai; howevei, usually the
whole pigmented aiea moves giadually foiwaid.
Distinction of ALM fiom subungual hemoi-
ihage can easily be made by deimoscopy. With
the destiuction of the nail plate, the lesions
aie most often iegaided as °fungal infection."
When nonpigmented tumoi nodules appeai,
they aie misdiagnosed as pyogenic gianuloma.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y The histologic diagnosis
of the iadial giowth phase of the volai type of
ALM may be difficult and may iequiie laige
incisional biopsies to piovide foi multiple sec-
tions. Theie is usually an intense lymphocytic
inflammation at the deimal-epideimal junc-
tion. Chaiacteiistic laige melanocytes along the
basal cell layei may extend as laige nests into the
deimis, along ecciine ducts. Invasive malignant
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 325
melanocytes aie often spindle shaped, so that
ALM fiequently has a desmoplastic appeaiance
histologically.
Fk0CN0SIS
The volai type of ALM can be deceptive in its
clinical appeaiance, and °flat" lesions may be
quite deeply invasive. Five-yeai suivival iates
aie <50%. The subungual type of ALM has
a bettei 5-yeai suivival iate (80%) than does
the volai type, but the data aie piobably not
accuiate. Pooi piognosis foi the volai type of
ALM may be ielated to inoidinate delay in the
diagnosis.
MANAC£M£NI
In consideiing suigical excision, it is impoitant
that the extent of the lesion be asceitained by
viewing the lesion with deimoscopy. Subun-
gual ALM and volai-type ALM: amputation
¦toe(s), fingei(s)]; volai and plantai ALM: wide
excision with split skin giafting. Sentinel lymph
node pioceduie necessaiy in most cases (see
°Management of Melanoma," page 332).
FICük£ 12-14 Acra| |eotì¿ìoous me|aooma â. Au A|\ +||º|uç ou ||e ||ur|. |eu||ç|uouº corpoueu| ou
||e do|º+| º||u o| ||e ||ur|. r+cu|+|, º|+|p|, +ud |||·de||ued ||oWu +ud ç|e,·||u|º| ºpo|º. 'u|uuçu+| +ud d|º|+|
u|ce|+|ed uodu|+| corpoueu|. 8. I|e |uro| |+º |ep|+ced ||e eu|||e u+|| |ed +ud ºu||ouud|uç º||u. r+cu|+| +ud o|
.+||eç+|ed co|o| |eºer|||uç + |eu||ço r+||çu+. I|e u+|| |+º |eeu º|ed. I||º |º A|\ ||+| |+º |ed |o deº||uc||ou
o| ||e u+|| r+|||\ +ud W+º |||º| d|+çuoºed +º u+|| d,º||op|,. C. A|\ ou ||e |ee|. I|e|e |º + ||ç||, .+||eç+|ed
r+cu|+| corpoueu|-||oWu |o ç|+, +ud ||+c|, ||e uodu|+| corpoueu| |º |,pe||e|+|o||c, |edd|º|, +ud u|ce|+|ed.
0. |eu||ço r+||çu+ re|+uor+ ou ||e ºo|e. I||º |º +u +d.+uced |eº|ou W||| + r+cu|+| corpoueu| +ud + |edd|º|,
u|ce|+|ed uodu|e. I|e |eº|ou re+ºu|ed ¹0 rr |u dep||, +ud ||e|e We|e eu|+|çed |uçu|u+| |,rp| uodeº.
â
C
8
0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 326
A|| |,peº o| re|+uor+ c+u |e +re|+uo||c.
'|uce ||e, dou'| |+.e ||e c|+|+c|e||º||c p|çreu|
r+||e| ||e, +|e + d|+çuoº||c c|+||euçe (||ç. ¹2·
¹5).
noWe.e|, o||eu ||e|e +|e p|çreu|ed c|oueº |u
||e |uro|, W||c| |e.e+| ||º u+|u|e +º + re|+uor+
(||çº. ¹2·¹53 +ud C).
|u roº| c+ºeº ou|, ||opº, W||| |e.e+| ||e co||ec|
d|+çuoº|º (||çº. ¹2·¹51 +ud |).
AM£IAN0IIC M£IAN0MA |C|·9 . 2!2
°
|C|·¹0 . |02
FICük£ 12-15 Ame|aootìc me|aooma â. Are|+uo||c |\\. I|e |ed uodu|e W+º ºo|| +ud d|+çuoºed +º
p,oçeu|c ç|+uu|or+ +ud W+º e\c|ºed. n|º|op+||o|oç, |e.e+|ed re|+uor+ +ud ºu|ºequeu| puuc| ||opº|eº pe|·
|o|red |u ||e e|,||er+|ouº º||u o| ||e c|ee| |e.e+|ed |eu||ço r+||çu+ (|\). I|e ou|||ueº o| ||e |\ |eº|ou +º
de|e|r|ued |, |u|||e| puuc| ||opº|eº +|e r+||ed W||| ç|eeu c||c|eº. |o|e ||+| o.e| ||e r+ud|||e |eº|ou |º +|ºo
uodu|+| (.e|||c+|) ç|oW||. 8. Are|+uo||c ºupe|||c|+| ºp|e+d|uç re|+uor+. I|e ||ue u+|u|e o| |||º |ed uodu|e |º
|e.e+|ed |, ||e ||ue c|eºceu| +| ||º |+ºe +ud ||e .+||eç+|ed ||oWu·|ed p|+que W||| W||c| || |º cou||çuouº.
C. Are|+uo||c uodu|+| re|+uor+. I||º c|e||,·|ed uodu|e |+º + ||oWu, r+cu|+| e\|euº|ou +| ¹2 +ud + o'c|oc|
+ud + ºecoud, ruc| ºr+||e| |ed uodu|e +| 9 o'c|oc|, ç|.|uç +W+, ||e co||ec| d|+çuoº|º. 0. Are|+uo||c A'\ ou ||e
|ee|. I||º c|e||,·|ed |eº|ou W+º c||u|c+||, d|+çuoºed +º ecc||ue po|or+. B|opº, |e.e+|ed deep|, |u.+d|uç A|\.
â
C
8
0
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 32T
\+||çu+u| re|+uor+º +||º|uç |u ||e rucoº+|
ep|||e||+| ||u|uç o| ||e |eºp||+|o|, ||+c| +ud ç+º·
||o|u|eº||u+| +ud çeu||ou||u+|, ||+c|º +|e .e|, |+|e,
W||| +u +uuu+| |uc|deuce o| 0.¹5° pe| ¹00,000
|ud|.|du+|º.
\+jo| º||eº o| ||e rucoº+| re|+uor+º +|e ||e
.u|.+ +ud .+ç|u+ (+5°) +ud ||e u+º+| +ud o|+|
c+.||, (+!°).
\ucoº+| re|+uor+º +|e ºo |+|e ||+| ||e|e +|e uo
|+|çe d+|+ |+ºeº corp+|ed |o ||oºe |o| cu|+ueouº
re|+uor+.
I|e|e|o|e, p+||o|oç|c r|c|oº|+ç|uç |+º uo| |eeu
poºº|||e, +ud ||e ||ue·|uu|uç o| ||e p|oçuoº|º ||+|
|+º |eeu uºe|u| |u cu|+ueouº re|+uor+ (B|eº|oW
|||c|ueºº) |+º ºo |+| uo| |eeu poºº|||e |u rucoº+|
re|+uor+.
MAIICNANI M£IAN0MA 0F Ih£ MüC0SA |C|·9 . 2!2
°
|C|·¹0 . |02
Me|aoomas oI the 0ra| Cavìty
Theie is a delay in diagnosis of melanoma of the
oial and nasal suifaces. Although melanosis of
the mucosa is common in blacks and East Indi-
ans, it involves the buccal and gingival mucosa
bilateially (see °Disoideis of the Mouth," Sec-
tion 34); when theie is a single aiea of melanosis
(see Fig. 34-13), a biopsy should be peifoimed
to iule out melanoma; this is also tiue of pig-
mented nevi in the oial cavity, which should be
excised (see Section 34).
Me|aoomas ìo the Ceoìta|ìa
These melanomas mostly aiise on the glans oi
piepuce (see Fig. 35-23) and the labia minoia;
theie aie fewei on the clitoiis and the labia
majoia (see Fig. 35-24). Most tumois extend to
the vagina at the mucocutaneous boidei. They
look and evolve like LM and LMM (see Figs.
35-23, 35-24). Vulva melanomas aie often flat
like LMM with laige aieas of melanoma in situ,
and this is impoitant to asceitain in planning
excision of all the lesion to pievent iecuiience.
Deimoscopy should be used to outline the
peiipheiy of the lesion, as is done in LMM
(see °Disoideis of the Genitalia, Peiineum, and
Anus," Section 35).
Aoorecta| Me|aooma
Often piesents with a localized, often polypoid
oi nodulai piimaiy tumoi, but it may also
piesent similaily to LMM.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 328
\e|+º|+||c re|+uor+ occu|º |u ¹5-2o° o| º|+çe |
+ud º|+çe || re|+uor+ (ºee |e|oW).
I|e ºp|e+d o| d|ºe+ºe ||or ||e p||r+|, º||e uºu+||,
occu|º |u + º|epW|ºe ºequeuce. p||r+|, re|+uor+
|eç|ou+| re|+º|+º|º (||ç. ¹2·¹1 3)
d|º|+u| re|+º|+º|º.
||º|+u| re|+º|+º|º c+u occu|, º||pp|uç ||e |e·
ç|ou+| |,rp| uodeº +ud |ud|c+||uç |er+|oç·
euouº ºp|e+d.
||º|+u| re|+º|+ºeº occu| +u,W|e|e |u| uºu+||,
|u ||e |o||oW|uç o|ç+uº. |uuçº (¹3-!o°), ||.e|
(¹+-29°), ||+|u (¹2-20°), |oue (¹¹-¹1°), +ud
|u|eº||ueº (¹-1°).
\oº| ||equeu||,, |oWe.e|, re|+uor+ |||º| ºp|e+dº
|o d|º|+u| |,rp| uodeº, º||u (||ç. ¹2·¹13), +ud
ºu|cu|+ueouº ||ººueº (+2 |o 51°) (||ç. ¹2·¹1|).
|oc+| |ecu||euce occu|º || e\c|º|ou |+º uo| |eeu
+dequ+|e (||ç. ¹2·¹o) o| || c+u |u.o|.e ||e º||u o|
+u eu|||e |eç|ou |o|| W||| +ud W|||ou| +dequ+|e
ºu|ç|c+| ||e+|reu| (||ç. ¹2·¹11 C )
w|deºp|e+d re|+º|+º|º c+u +|ºo |e+d |o º|uç|e
re|+º|+||c re|+uor+ ce|| |odçereu| |u +|| o|ç+uº
W||| re|+uoº|º o| ||e º||u (||ç. ¹2·¹3), rucouº
rer||+ueº, ||.e|, ||due,, |e+|| ruºc|e +ud o||e|
||ººueº.
!-|cº|c|·: ¤-|c¤¤¤c +·||¤±| c ¤··¤c·, |±¤¤·
|º |+|e, ¹-o°. || |º ||e |eºu|| o| re|+º|+º|º ||or
+ re|+uor+ ||+| uude|Weu| |o|+| ºpou|+ueouº
|eç|eºº|ou.
!-|c¤¤¤c ¤c, |c.- c |c|- ·-:±··-¤:- ( ¹0
,e+|º). I|e uºu+| ||re |º ¹+ ,e+|º, |u| ||e|e |+.e
|eeu '.e|, |+|e¨ |ecu||euceº (>¹5 ,e+|º) |u oue
ºe||eº +| ||e \+ºº+c|uºe||º Ceue|+| noºp||+|, W|||
0.012° (20 o| 21oo C+ºeº).
|c|·-¤|º +·|| c º¤|·|c·, ¤-|cº|cº·º cou||ued |o ||e
ºu|cu|+ueouº, uou|eç|ou+| |,rp| uodeº o| |uuç
+|e roº| |||e|, |o |eue||| ||or ºu|ç|c+| |u|e|.eu·
||ou.
M£IASIAIIC M£IAN0MA
FICük£ 12-16 Metastatìc me|aooma: recurrìo¿ ìo excìsìoo scar â. A p|çreu|ed |eº|ou ou ||e º||u o| +
!5·,e+|·o|d r+|e, p|eºeu| |o| < 2 ,e+|º. |e|r+|op+||o|oç, W+º |u|||+||, |u|e|p|e|ed +º + ºp|ud|e ce|| ('p||/) ue.uº.
I|e p||r+|, |eº|ou º||e W+º ||e|e|o|e uo| |ee\c|ºed. 8. IWo p+pu|eº +|e ºeeu +|ouud ||e e\c|º|ou º||e ºc+|, oue
o| W||c| |+º + ||ue·||oWu co|o|. I|e ||º|o|oç, ||or ||e e\c|ºed |eº|ou W+º |e.|eWed +ud |e.|ºed +º + ºupe|||c|+|
ºp|e+d|uç re|+uor+, +ud ||e ||º|op+||o|oç, o| ||e |Wo p+pu|eº ºeeu |e|e W+º re|+º|+||c re|+uor+.
â 8
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 329
â 8
FICük£ 12-1T Metastatìc me|aooma â. |oc+| |ecu||euce +ud |u·||+uº|| cu|+ueouº re|+º|+ºeº +||e| e\c|º|ou
o| p||r+|, re|+uor+ ou ||e ºc+|p +ud ºp||| º||u ç|+|||uç. |o|e. re|+º|+ºeº +|e |o|| |u ||e ºu||ouud|uç º||u +ud
||e ç|+||. 8. Ad.+uced re|+º|+ºeº |u ||e +\|||+|, |,rp| uodeº +ud |u·||+uº|| re|+º|+ºeº o| ||e r+rr+|, º||u. I|e
p||r+|, |uro| |+d |eeu + p||c|·||+c| uodu|+| re|+uor+ +ud |+d |eeu juº| |+|e|+| |o ||e ||e+º| (||e ºc+| c+u
º|||| |e ºeeu). |o|e ||+| |o|| ||e |u·||+uº|| +ud +\|||+|, uodu|eº e\|eud|uç |u|o ||e º||u +|e +re|+uo||c. C. \u|||p|e
re|+uor+ re|+º|+ºeº |o ||e º||u +||e| |er+|oçeuouº ºp|e+d. 0. 'u|cu|+ueouº re|+uor+ re|+º|+ºeº |, |er+·
|oçeuouº ºp|e+d. '|uce ||e, +|e uo| ||u|º| ||e, +|e +re|+uo||c. |||r+|, +ud re|+º|+||c re|+uor+ r+, d|||e| W|||
|eç+|d |o p|çreu|+||ou po|eu||+|.
C 0
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 330
â
FICük£ 12-18 üoìversa|
me|aoosìs due to metastatìc
me|aooma â. '|uç|e·ce||
re|+º|+ºeº +|e |ouud |||ouç|·
ou| ||e º||u +ud rucouº rer·
||+ueº o| ||e W|||e p+||eu| +ud
c||cu|+||uç re|+º|+||c re|+uor+
ce||º We|e |ouud |u ||e ||ood.
I|e u||ue W+º ||+c| (re|+uo·
çeuu||+), +ud upou +u|opº, ||e
|u|e|u+| o|ç+uº We|e +|ºo ||+c|.
8. I|e p+||eu|'º |+ud |º º|oWu
|eº|de ||e |+ud o| + uu|ºe |o
derouº||+|e ||e d|||e|euce |u
co|o|.
8
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 331
Mìcrosta¿ìo¿
Mítrosìagíng is done accoiding to Bieslow method.
The thickness of the piimaiy melanoma is
measuied fiom the gianulai layei of the epi-
deimis to the deepest pait of the tumoi. The
thickness of melanoma (level of invasion) is the
most impoitant single piognostic vaiiable and
thus decisive foi theiapeutic decisions (Tables
12-6, 12-7).
Claik miciostaging accoiding to tissue level
of invasion is no longei consideied a significant
piognostic vaiiable.
Seotìoe| Iymph Node 8ìopsy
Sentinel lymph node biopsy can piedict the
piesence of clinically nondetectable metastatic
melanoma within iegional lymph nodes with
the identification of malignant cells in H&E
sections; staining foi S-100 piotein, HMB-45,
and tyiosinase.
When the nodes aie not palpable, it is not
ceitain if theie aie miciometastases; these can
be detected by the senìíne| noJe ìet|níque . The
hypothesis is that the [írsì node diaining a
lymphatic basin, called the senìíne| noJe , can
piedict the piesence oi absence of metastasis
in othei nodes in that basin. Eithei lymphatic
mapping (LM) oi sentinel lymphadenectomy
(SL) is peifoimed on the same day with a single
injection of filteied
99m
Tc subcutaneously into
the site of the piimaiy melanoma foi piobe-
diiected LM and SL. Alteinatively, one day
aftei lymphoscintigiaphy, sentinel node biopsy
is peifoimed, guided by a gamma piobe and
blue dye also injected into the piimaiy site; the
sentinel node is subjected to histopathology and
immunohistochemistiy. LM is veiy useful in lo-
cating the diainage aieas, especially in piimaiy
Claik level I, intiaepideimal; level II, invades papil-
laiy deimis; level III, fills papillaiy deimis; level IV,
invades ieticulai deimis; level V, invades subcutane-
ous fat.
Claik level I, intiaepideimal; level II, invades papil-
laiy deimis; level III, fills papillaiy deimis; level IV,
invades ieticulai deimis; level V, invades subcutane-
ous fat.
'|+ç|uç o| re|+uor+ depeudº ou ||º I|\ C|+º·
º|||c+||ou (p||r+|, |uro|, |eç|ou+| ¤ odeº, ¤ e|+º·
|+ºeº, I+||e ¹2·1).
C|·¤·:c| º|c¸·¤¸ o| re|+uor+ d|||e|eu||+|eº |e·
|Weeu |oc+|, |eç|ou+|, +ud d|º|+u| d|ºe+ºe +ud
|º |+ºed ou r|c|oº|+ç|uç o| ||e re|+uor+ +ud
c||u|c+| +ud |r+ç|uç e.+|u+||ou |o| re|+º|+ºeº.
|c||¤|¤¸·: º|c¸·¤¸ couº|º|º o| r|c|oº|+ç|uç o|
||e p||r+|, |uro| +ud p+||o|oç|c e.+|u+||ou o|
|eç|ou+| |,rp| uodeº (I+||eº ¹2·5 +ud ¹2·o).
'|+ç|uç o| re|+uor+ |º º||ouç|, co||e|+|ed W|||
ºu|.|.+|.
SIACINC 0F M£IAN0MA
tumois on the tiunk, which can diain on eithei
side and to both the axillaiy and inguinal lymph
nodes.
Lymph node dissection is peifoimed only if
miciometastasis is found in the sentinel node.
The sentinel node technique is also essential
in making a decision about the use of adjuvant
theiapy.
W0kküF 0F M£IAN0MA
I Piimaiy melanoma: Stage I oi II (no nodes
palpated)
A. Chest ioentgenogiam, sonogiaphy of
lymph nodes
B. Livei function tests, LDH
C. Lymphatic mapping and sentinel lym-
phadenectomy in stage I thickness >1.0
mm
II Piimaiy melanoma with local-iegional dis-
ease
A. Stage III, satellites and local iecuiience
1. Complete blood count
2. Livei function tests, LDH
3. Chest ioentgenogiam
4. Ultiasound and CT scans: abdomen,
pelvis (with disease below the waist),
neck (with disease in the head and
neck); position emission tomogia-
phy (PET) scan
B. Stage IV
1. Same as foi stage III
2. CT scan of the chest
3. MRI of the biain
4. Bone scan
5. GI seiies (on the basis of symptoms)
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 332
CüI0£IIN£S F0k 8I0FS¥ AN0 SükCICAI
Ik£AIM£NI 0F FAII£NIS WIIh M£IAN0MA
I. Biopsy
A. Total excisional biopsy with naiiow
maigins-optimal biopsy pioceduie,
wheie possible.
B. Incisional oi punch biopsy acceptable
when total excisional biopsy cannot be
peifoimed oi when lesion is laige, ie-
quiiing extensive suigeiy to iemove the
entiie lesion.
C. When sampling the lesion: If iaised,
iemove the most iaised aiea; if flat, ie-
move the daikest aiea.
II. Melanoma in situ
A. Excise with 0.5-cm maigin.
III. Lentigo maligna melanoma
A. Excise with a 1-cm maigin beyond the
clinically visible lesion oi biopsy scai-
unless the flat component involves a
majoi oigan (e.g., the eyelid), in which
case lessei maigins aie acceptable.
B. Excise down to the fascia oi to the un-
deilying muscle wheie fascia is absent.
Skin flaps oi skin giafts may be used foi
closuie.
C. No node dissection is iecommended
unless nodes aie clinically palpable and
suspicious foi tumoi.
D. See iecommendation foi sentinel
node studies foi thickness >1 mm
(page 331).
||oçuoº|º o| re|+uor+ c+u |e e|||e| e\ce||eu|
o| ç|+.e, depeud|uç ou W|e||e| ||e |uro|
|º d|+çuoºed e+||, o| |+|e, W|eu |eç|ou+| o|
d|º|+u| re|+º|+ºeº |+.e occu||ed (I+||e ¹2·5).
I||º erp|+º|/eº ||e |rpo||+uce o| e+||, d|+ç·
uoº|º, o| queº||ou|uç p+||eu|º |o| re|+uor+
||º|º (I+||eº ¹2·2 +ud ¹2·!), o| ºc|eeu|uç
|ud|.|du+|º |e|ouç|uç |o ||º| ç|oupº, +ud o|
|o|+|·|od, e\+r|u+||ou o| +u, p+||eu| ºee|uç
+ p|,º|c|+u |o| red|c+| e\+r|u+||ou. ||oçuo·
º|º |e|+||uç |o º|+çe ç|oup|uç |o| cu|+ueouº
re|+uor+ |º º|oWu |u I+||e ¹2·5.
Fk0CN0SIS 0F M£IAN0MA
I|e ou|, cu|+||.e ||e+|reu| o| re|+uor+ |º e+||, ºu|ç|c+| e\c|º|ou.
MANAC£M£NI 0F M£IAN0MA
IV. Supeificial spieading melanoma, nodulai
melanoma, and acial lentiginous melanoma
A. Thickness <1 mm
1. Excise with a 1-cm maigin fiom the le-
sion edge.
2. Excise down to the fascia oi to the un-
deilying muscle wheie fascia is absent.
Diiect closuie without giaft is often
possible.
3. Node dissection is not iecommended
unless nodes aie clinically palpable and
suspicious foi tumoi.
B. Thickness 1-4 mm
1. Excise 2 cm fiom the edge of the lesion,
except on the face, wheie naiiowei mai-
gins may be necessaiy.
2. Excise down to the fascia oi to the un-
deilying muscle wheie fascia is absent.
Giaft may be iequiied.
3. The sentinel node pioceduie foi tumois
with thickness >1 mm is iecommended.
4. Lymphadenectomy is selectively pei-
foimed and only foi those nodal basins
with occult tumoi cells (i.e., positive
sentinel lymph node). If the sentinel
node is negative, then the patient is
spaied a lymph node dissection.
5. Theiapeutic nodal dissection is iecom-
mended if nodes aie clinically palpable
and suspicious foi tumoi.
6. If iegional node is positive and com-
pletely iesected with no evidence of
distant disease, adjuvant theiapy with in-
teifeion- -2b (IFN- -2b) is consideied.
S£CII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 333
A0lüvANI Ih£kAF¥
This is tieatment of a patient aftei iemoval
of all detectable tumoi but the patient is con-
sideied at high iisk foi iecuiience (i.e., stages
IIb and III). As mentioned above, IFN- -2b
(both high and low dose) is subject to intensive
investigation; howevei, despite eaily piomising
iesults to date no cleai benefit on oveiall sui-
vival has been convincingly demonstiated.
Maoa¿emeot oI 0ìstaot Metastases (Sta¿e Iv)
Cuiiently this can be consideied palliative at
best. Suigical iemoval of accessible metastases
can piovide excellent palliation. Chemotheiapy
encompasses a laige list of diugs (dacaibazine/
temozolomide, cisplatin, vindesine/vinblastine,
fotemustine, taxol/taxoteie) employed as single
agents oi in combination. Dacaibazine is still
the most effective monotheiapeutic agent, but
all in all chemotheiapeutic tieatment of stage
IV melanoma is disappointing, showing only
a 20% iesponse iate and no effect on oveiall
suivival. Theie aie a laige numbei of melanoma
vaccination tiials piesently being peifoimed,
and the field is iapidly expanding to include
gene-theiapeutic appioaches such as anti-Bcl-
2 oligonucleotide theiapy. Inciease of oveiall
suivival has, howevei, not been shown to date.
Radiotheiapy has only palliative effects also,
but steieotactive iadiosuigeiy with the gamma-
knife has shown consideiable palliation.
F0II0W-üF F0k FkIMAk¥ M£IAN0MA
See Table 12-8.
IA8I£ 12-8 |o||ow·up for Primary Ne|anoma
Stages | (>1 mm) aod ||, Stage |||, Lymph
Stage | (<1 mm) Lymph hodes hegat|ve hodes Pos|t|ve
E.e|, !-o rou||º
c
E.e|, !-o rou||º
c
|o| E.e|, !-o rou||º |o| !
|o| ! ,e+|º ! ,e+|º ,e+|º, ||eu !-¹2 rou||º
|o| 2 ,e+|º
ke.|eW o| º,º|erº ke.|eW o| º,º|erº ke.|eW o| º,º|erº
||,º|c+| e\+r|u+||ou ||,º|c+| e\+r|u+||ou ||,º|c+| e\+r|u+||ou
||.e| |uuc||ou (||n) CBC, ||.e| |uuc||ou (||n)
C|eº| \·|+, +ud CI ºc+uº C|eº| \·|+, +ud CI
e.e|, o rou||º ºc+uº e.e|, o rou||º
Auuu+| e\+r|u+||ou Auuu+| e\+r|u+||ou Auuu+| e\+r|u+||ou
|o| |||e |o| |||e |o| |||e
c
|+r|||+| re|+uor+ d,ºp|+º||c ue.uº º,ud|ore. e.e|, ! rou||º |o| ! ,e+|º, +ud ||eu e.e|, o rou||º |o| 5 ,e+|º, +ud ||eu +uuu+||, |o| |||e.
334
S E C I | 0 N 1 5
vIIIIIC0
FICM£NIAk¥ 0IS0k0£kS
|o|r+| º||u co|o| |º corpoºed o| + r|\|u|e o|
|ou| ||oc||oreº, u+re|,, (¹) ·-!±:-! |-¤¤·
¸|¤|·¤ (||ue), (2) ¤·,|-¤¤¸|¤|·¤ (|ed), (!)
:c·¤|-¤¤·!º (,e||oW, e\oçeuouº ||or d|e|), +ud
(+) ¤-|c¤·¤ (||oWu).
I|e p||uc|p+| de|e|r|u+u| o| ||e º||u co|o| |º
re|+u|u p|çreu|, +ud .+||+||ouº |u ||e +rouu|
+ud d|º||||u||ou o| re|+u|u |u ||e º||u +|e ||e
|+º|º o| ||e |||ee p||uc|p+| |ur+u º||u co|o|º.
||+c|, ||oWu, +ud W|||e.
I|eºe |||ee |+º|c º||u co|o|º +|e çeue||c+||,
de|e|r|ued +ud +|e c+||ed :¤¤º|·|±|·.- ¤-|c·
¤·¤ ¤·¸¤-¤|c|·¤¤ , ||e uo|r+| |+º|c º||u co|o|
p|çreu|+||ou c+u |e |uc|e+ºed de|||e|+|e|, |,
e\poºu|e |o u|||+.|o|e| |+d|+||ou (u\k) o| p||u||+|,
|o|roueº, +ud |||º |º c+||ed ·¤!±:·||- ¤-|c¤·¤
¤·¸¤-¤|c|·¤¤ .
I|e cor||u+||ou o| ||e couº|||u||.e +ud |uduc|||e
re|+u|u p|çreu|+||ou de|e|r|ueº W|+| |º c+||ed
||e º|·¤ ¤|¤|¤|,¤- ('|I) (ºee I+||e ¹0-2). E||u|c·
||, |º uo| ueceºº+|||, + p+|| o| ||e de||u|||ou, e.ç.,
A|||c+u '||+c|¨ e||u|c pe|ºouº c+u |e '|I ||| +ud
+u E+º| |ud|+u C+uc+º|+u c+u |e '|I |\ o| e.eu
\. í|- º|·¤ ¤|¤|¤|,¤- ·º c ¤c·|-· |¤· º|·¤ :c¤:-·
··º| c¤! º|¤±|! |- ·-:¤·!-! c| ||- |··º| ¤c|·-¤|
.·º·| .
|uc|e+ºe o| re|+u|u |u ||e ep|de|r|º |eºu||º |u +
º|+|e |uoWu +º |,¤-·¤-|c¤¤º·º. I||º |e||ec|º oue
o| |Wo |,peº o| c|+uçeº.
Au |uc|e+ºe |u ||e uur|e| o| re|+uoc,|eº |u ||e
ep|de|r|º p|oduc|uç |uc|e+ºed |e.e|º o| re|+u|u,
W||c| |º c+||ed ¤-|c¤¤:,|¤|·: |,¤-·¤-|c¤¤º·º (+u
e\+rp|e |º |-¤|·¸¤).
|¤ |uc|e+ºe o| re|+uoc,|eº |u| +u |uc|e+ºe
|u ||e p|oduc||ou o| re|+u|u ou|,, W||c| |º
c+||ed ¤-|c¤¤|·: |,¤-·¤-|c¤¤º·º (+u e\+rp|e |º
¤-|cº¤c ).
n,pe|re|+uoº|º o| |o|| |,peº c+u |eºu|| ||or
|||ee |+c|o|º. çeue||c, |o|rou+| (+º |u Add|ºou
d|ºe+ºe), W|eu || |º c+uºed |, +u |uc|e+ºe |u
c||cu|+||uç p||u||+|, re|+uo||op|c |o|roueº, +ud
u\k (+º |u |+uu|uç).
n,pore|+uoº|º |º + dec|e+ºe o| re|+u|u |u ||e
ep|de|r|º. I||º |e||ec|º r+|u|, |Wo |,peº o|
c|+uçeº.
|o dec|e+ºe o| re|+uoc,|eº |u| + dec|e+ºe o|
||e p|oduc||ou o| re|+u|u ou|, ||+| |º c+||ed
¤-|c¤¤¤-¤·: |,¤¤¤-|c¤¤º·º (+u e\+rp|e |º
+|||u|ºr).
A dec|e+ºe |u ||e uur|e| o| +|ºeuce o|
re|+uoc,|eº |u ||e ep|de|r|º p|oduc|uç uo
o| dec|e+ºed |e.e|º o| re|+u|u. I||º |º c+||ed
¤-|c¤¤:,|¤¤-¤·: |,¤¤¤-|c¤¤º·º (+u e\+rp|e
|º .|||||ço).
n,pore|+uoº|º +|ºo |eºu||º ||or çeue||c (+º |u
+|||u|ºr), ||or +u|o|rruue (+º |u .|||||ço), o|
o||e| |u||+rr+|o|, p|oceººeº (+º |u poº||u||+r·
r+|o|, |eu|ode|r+ |u pºo||+º|º).
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 335
£FI0£MI0I0C¥
Sex Equal in both sexes. The piedominance in
women suggested by the liteiatuie likely ieflects
the gieatei concein of women about cosmetic
appeaiance.
A¿e oI 0oset May begin at any age, but in
50% of cases it begins between the ages of 10
and 30 yeais. A few cases have been iepoited to
be piesent at biith; onset in old age also occuis
but is unusual.
Iocìdeoce Common, woildwide. Affects up to
1% of the population.
kace All iaces. The appaiently incieased piev-
alence iepoited in some countiies and among
daikei-skinned peisons iesults fiom a diamatic
contiast between white vitiligo macules and
daik skin and fiom maiked social stigma in
countiies such as India, wheie even today the
oppoitunities foi advancement oi maiiiage
among affected individuals aie limited.
Ioherìtaoce Vitiligo has a genetic backgiound;
>30% of affected individuals have iepoited
vitiligo in a paient, sibling, oi child. Vitiligo
in identical twins has been iepoited. Tians-
mission is most likely polygenic with vaiiable
expiession. The iisk of vitiligo foi childien of
affected individuals is unknown but may be
<10%. Individuals fiom families with an in-
cieased pievalence of thyioid disease, diabetes
mellitus, and vitiligo appeai to be at incieased
iisk foi development of vitiligo.
FAIh0C£N£SIS
Thiee piincipal theoiies have been piesented
about the mechanism of destiuction of melano-
cytes in vitiligo:
1. The auìoímmune ì|eory holds that se-
lected melanocytes aie destioyed by ceitain
lymphocytes that have somehow been
activated.
wo||dW|de occu||euce, ¹° o| popu|+||ou
+||ec|ed.
A r+jo| pº,c|o|oç|c+| p|o||er |o| ||oWu o| ||+c|
pe|ºouº, |eºu|||uç |u ºe.e|e d||||cu|||eº |u ºoc|+|
+djuº|reu|.
A c||ou|c d|ºo|de| W||| ru||||+c||ou+| p|ed|ºpoº|·
||ou +ud |||ççe||uç |+c|o|º.
C||u|c+||, c|+|+c|e||/ed |, |o|+||, W|||e r+cu|eº,
W||c| eu|+|çe +ud c+u +||ec| ||e eu|||e º||u.
\|c|oºcop|c+||,. corp|e|e +|ºeuce o| re|+uo·
c,|eº.
Aººoc|+|ed W||| º,º|er|c +u|o|rruue +ud/o|
eudoc||ue d|ºe+ºe.
vIIIIIC0 |C|·9 . 109.0¹
°
|C|·¹0 . |30
2. The neurogenít |y¡oì|esís is based on an
inteiaction of the melanocytes and neive
cells.
3. The se|[-Jesìrutì |y¡oì|esís suggests that
melanocytes aie destioyed by toxic sub-
stances foimed as pait of noimal melanin
biosynthesis.
While the immediate mechanism foi the
evolving white macules involves piogiessive
destiuction of selected melanocytes by cytotoxic
T cells, othei genetically deteimined cytobio logic
changes and cytokines must be involved. Vitiligo
may follow cytokine deimatitis aftei imiquimod.
Because of diffeiences in the extent and couise
of segmental and geneialized vitiligo, the patho-
genesis of these two types is piobably diffeient.
CIINICAI MANIF£SIAII0N
Many patients attiibute the onset of theii
vitiligo to physical tiauma (wheie vitiligo
appeais at the site of tiauma-Koebnei phe-
nomenon), illness, oi emotional stiess. Onset
aftei the death of a ielative oi aftei seveie
physical injuiy is often mentioned. A sunbuin
ieaction may piecipitate vitiligo.
Skìo Iesìoos Macules, 5 mm to 5 cm or
more in diametei (Figs. 13-1 and 13-2).
°Chalk" oi pale white, shaiply maiginated.
The disease piogiesses by giadual enlaigement
of the old macules oi by development of new
ones. Maigins aie ton·ex (as if the pathologic
piocess of de pigmentation weie flowing into
noimally pigmented skin).Tiichiome vitiligo
(thiee colois: white, light biown, daik biown)
iepiesents diffeient stages in the evolution of
vitiligo. Newly developed macules may be °off-
white" in coloi; this also iepiesents a tiansitional
phase. Pigmentation aiound a haii follicle in a
white macule iepiesents iesidual pigmentation
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 336
oi ietuin of pigmentation (Fig. 13-3). Confetti-
sized hypomelanotic macules may also be
obseived. In[|ammaìory ·íìí|ígo has an elevated
eiythematous maigin and may be piuiitic.
DIstrIhutIvn Two geneial patteins. The [ota|
type is chaiacteiized by one oi seveial macules
in a single site; this may be an eaily evolution-
aiy stage of one of the othei types in some
cases. Cenera|í:eJ vitiligo is moie common and
is chaiacteiized by widespiead distiibution of
depigmented macules, often in a iemaikable
symmetiy (Fig. 13-2). Typical macules occui
aiound the eyes (Fig. 13-1) and mouth and
on digits, elbows, and knees, as well as on the
low back and in genital aieas (Image 13-1).
The ° |í¡-ìí¡ " ¡aììern involves the skin aiound
the mouth as well as on distal fingeis and
toes; lips, nipples, genitalia (Fig. 35-6, tip of
the penis), and anus may be involved. Conflu-
ence of vitiligo iesults in laige white aieas, and
extensive geneialized vitiligo may leave only a
few noimally pigmented aieas of skin- ·íìí|ígo
uní·ersa|ís (Fig. 13-4).
Se¿meota| vìtì|ì¿o This is a special subset
that usually develops in one unilateial iegion;
usually does not extend beyond that initial
onesided iegion (though not always); and, once
piesent, is veiy stable. May be associated with
vitiligo elsewheie.
Assocìated Cutaoeous Fìodìo¿s White haii and
piematuiely giay haii. Ciicumsciibed aieas of
white haii, analogous to vitiligo macules, aie
called ¡o|íosís . Alopecia aieata and halo nevi.
In oldei patients, photoaging as well as solai
keiatoses may occui in vitiligo macules in those
with histoiy of long exposuies to sunlight.
Squamous cell caicinoma, limited to the white
macules, has iaiely been iepoited.
Ceoera| £xamìoatìoo Not uncommonly as-
sociated with thyioid disease (up to 30% of all
vitiligo cases: Hashimoto thyioiditis, Giaves
disease); also diabetes mellitus-piobably <5%;
peinicious anemia (uncommon, but incieased
iisk); Addison disease (uncommon); and multi-
ple endociinopathy syndiome (iaie). Ophthal-
mologic examination may ieveal evidence of
healed choiioietinitis oi iiitis (piobably <10%
of all cases). Vision is unaffected. Heaiing is
noimal. The Vogì-Koyanagí-HaraJa synJrome
is vitiligo - poliosis - uveitis - dysacusis -
alopecia aieata.
IA80kAI0k¥ £XAMINAII0NS
Wood Iamp £xamìoatìoo This examination
is iequiied to evaluate macules, paiticulaily in
lightei skin types, and to identify macules in
sun-piotected aieas in all but the daikest skin
types.
0ermatopatho|o¿y In ceitain difficult cases,
a skin biopsy may be iequiied. Established
vitiligo macules show noimal skin except foi
an absence of melanocytes. Use special stains
to identify melanocytes. Theie may be a mild
lymphocytic iesponse. These changes aie not
diagnostic foi vitiligo, howevei, only consistent
with it.
£|ectroo Mìcroscopy Absence of melanocytes
and of melanosomes in keiatinocytes; also
changes in keiatinocytes: spongiosis, exocytosis,
basilai vacuopathy, and apoptosis. Lymphocytes
have been seen in the epideimis.
Iaboratory Studìes Thyioxine (T
4
), thyioid-
stimulating hoimone (iadioimmunoassay),
fasting blood glucose, complete blood count
with indices (peinicious anemia), ACTH stimu-
lation test foi Addison disease, if suspected.
0IACN0SIS
Noimally, diagnosis of vitiligo can be made
ieadily on clinical examination of a patient with
piogiessive, acquiied, chalk-white, bilateial
(usually symmetiic), shaiply defined macules
in typical sites.
0IFF£k£NIIAI 0IACN0SIS 0F vIIIIIC0
|·|,··cº·º c||c (º||ç|| ºc+||uç, |u//, r+|ç|uº, o||·
W|||e co|o|).
|·|,··cº·º .-·º·:¤|¤· c||c (||ue ºc+|eº W||| ç|eeu·
|º|·,e||oW ||uo|eºceuce uude| wood |+rp, poº|||.e
K0n.
C|-¤·:c| |-±|¤!-·¤c (||º|o|, o| e\poºu|e |o ce|·
|+|u p|euo||c çe|r|c|deº, cou|e||| r+cu|eº). I||º |º
+ d||||cu|| d|||e|eu||+| d|+çuoº|º, +º re|+uoc,|eº +|e
+|ºeu| +º |u .|||||ço.
|-¤·¤º, (euder|c +|e+º, o||·W|||e co|o|, uºu+||, |||·
de||ued c¤-º||-|·: r+cu|eº).
|-.±º !-¤·¸¤-¤|¤º±º (º|+||e, couçeu||+|, o||·W|||e
r+cu|eº, uu||+|e|+|).
|,¤¤¤-|c¤¤º·º ¤| ||¤ ¦|||+|e|+|, B|+ºc||o ||ueº,
r+|||e c+|e p+||e|u, o0-15° |+.e º,º|er|c |u·
.o|.ereu|-ceu||+| ue|.ouº º,º|er (C|'), e,eº,
ruºcu|oº|e|e|+| º,º|er).
|-.±º c¤-¤·:±º (doeº uo| eu|+uce W||| wood
|+rp, doeº uo| º|oW e|,||er+ +||e| |u|||uç).
í±|-·¤±º º:|-·¤º·º (º|+||e, couçeu||+| o||·W|||e r+c·
u|eº po|,çou+|, +º|·|e+| º|+pe, · occ+º|ou+| ºeçreu·
|+| r+cu|eº, +ud cou|e||| r+cu|eº).
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 33T
FICük£ 13-1 vìtì|ì¿o: Iace E\|euº|.e dep|çreu|+||ou o| ||e ceu||+| |+ce. |u.o|.ed .|||||ç|uouº º||u |+º cou·
.e\ |o|de|º, e\|eud|uç |u|o ||e uo|r+| p|çreu|ed º||u. |o|e ||e c|+||W|||e co|o| +ud º|+|p r+|ç|u+||ou. |¤|-
c|º¤ ||c| ||- !-·¤c| ¤-.¤¤-|c¤¤:,|·: ¤-.±º ¤¤ ||- ±¤¤-· |·¤ |cº ·-|c·¤-! ·|º ¤·¸¤-¤|c|·¤¤
FICük£ 13-2 vìtì|ì¿o: koees |ep|çreu|ed, º|+|p|, der+|c+|ed r+cu|eº ou ||e |ueeº. Ap+|| ||or ||e |oºº
o| p|çreu|, .|||||ç|uouº º||u +ppe+|º uo|r+|. I|e|e |º º|||||uç º,rre||,. |o|e ||u, |o|||cu|+| p|çreu|ed ºpo|º W||||u
||e .|||||ço +|e+º ||+| |ep|eºeu| |ep|çreu|+||ou.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 338
|·-|c|!·º¤ (couçeu||+|, W|||e |o|e|oc|, º|+||e, do|º+|
p|çreu|ed º|||pe ou |+c|, d|º||uc||.e p+||e|u W|||
|+|çe |,pe|p|çreu|ed r+cu|eº |u ||e ceu|e| o| ||e
|,pore|+uo||c +|e+º).
|-±|¤!-·¤c cºº¤:·c|-! +·|| ¤-|c¤¤¤c (r+, uo|
|e ||ue .|||||ço |u+ºruc| +º re|+uoc,|eº, +|||ouç|
|educed, +|e uºu+||, p|eºeu|).
|¤º|·¤||c¤¤c|¤·, |-±|¤!-·¤c ¦o||·W|||e r+cu|eº
(uºu+||, + ||º|o|, o| pºo||+º|º o| ec/er+ |u ||e º+re
r+cu|+| +|e+, ºee ||ç. ¹!·¹+), |upuº e|,||er+|oºuº
uo| ºo º|+|p|,, de||ued|.
!,:¤º·º |±¤¸¤·!-º (r+, |e cou|uº|uç +º ou|,
dep|çreu|+||ou r+, |e p|eºeu| +ud ||opº, |º uec·
eºº+|,).
l¤¸|·|¤,c¤c¸··|c·c!c º,¤!·¤¤- (.|º|ou p|o||erº,
p|o|op|o||+, |||+|e|+| d,º+cuº|º).
|cc·!-¤|±·¸ º,¤!·¤¤- (corroueº| c+uºe o| cou·
çeu||+| de+|ueºº, W|||e r+cu|eº +ud W|||e |o|e|oc|,
|||º |e|e|oc||or|+).
C0ükS£ AN0 Fk0CN0SIS
Vitiligo is a chionic disease. The couise is
highly vaiiable, but iapid onset followed by
a peiiod of stability oi slow piogiession is
most chaiacteiistic. Up to 30% of patients may
iepoit some spontaneous iepigmentation in a
few aieas-paiticulaily aieas that aie exposed
to the sun. Rapidly piogiessive, oi °galloping,"
vitiligo may quickly lead to extensive depig-
mentation with a total loss of pigment in skin
and haii, but not eyes.
The tieatment of vitiligo-associated disease (i.e.,
thyioid disease) has no impact on the couise of
vitiligo. Suipiisingly, theie is less than expected
numbei of solai keiatoses, SCCIS, invasive SCC
oi BCE in vitiligo spots.
MANAC£M£NI
The appioaches to the management of vitiligo
aie as follows:
Suoscreeos
The dual objectives of sunscieens aie piotection
of involved skin fiom acute sunbuin ieaction
and limitation of tanning of noimally pig-
mented skin. Sunscieens with a sun piotection
factoi >30 aie ieasonable choices to pievent
sunbuin foi most patients and to limit the
tanning ieaction in faiiei-skinned individuals.
While all SPTs have a need foi sun piotection,
sunscieens alone aie often peifectly adequate
management foi those vitiligo patients with
SPTs I, II, and sometimes III.
Cosmetìc Coverup
The objective of coveiup with dyes oi makeup
is to hide the white macules so that the vitiligo
FICük£ 13-3 vìtì|ì¿o repì¿meotatìoo A |o|||cu|+| p+||e|u o| |ep|çreu|+||ou due |o |u\A ||e|+p, occu|||uç
|u + |+|çe .|||||ç|uouº r+cu|e ou ||e |oWe| +|doreu. \e|+uoc,|eº r+, pe|º|º| |u ||e |+|| |o|||c|e ep|||e||ur +ud
ºe|.e |o |epopu|+|e |u.o|.ed º||u, ºpou|+ueouº|, o| W||| p|o|oc|ero||e|+p,.
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 339
FICük£ 13-4 üoìversa| vìtì|ì¿o \|||||ç|uouº r+cu|eº |+.e co+|eºced |o |u.o|.e +|| º||u º||eº W||| corp|e|e
dep|çreu|+||ou o| º||u +ud |+|| |u + |er+|e. I|e p+||eu| |º We+||uç + ||+c| W|ç +ud |+º d+||eued ||e ||oWº W|||
e,e||oW peuc|| +ud e,e||d r+|ç|uº W||| e,e ||ue|.
IMAC£ 13-1 vìtì|ì¿o: p|ed||ec·
||ou º||eº.
Hypomelanosis
of hair
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 340
is not appaient. Ovei-the-countei piepaiations
come in many coloi shades, aie easy to apply,
and do not iub off but giadually wash oi weai
off. So-called self-tanning agents, which contain
dihydioxyacetone, aie available in a numbei of
foimulations.
kepì¿meotatìoo
The objective of iepigmentation (Figs. 13-3
and 13-5) is the peimanent ietuin of noimal
melanin pigmentation. This may be achieved
foi local macules with topical glucocoiticoids oi
topical psoialens and UVA (long-wave ultiavio-
let light) and foi widespiead macules with oial
psoialens and UVA (PUVA).
· To¡íta| g|utotorìítoíJs Initial tieatment with
inteimittent (4 weeks on, 2 weeks off) topical
class I glucocoiticoid ointments is piactical,
simple, and safe foi single oi a few macules. If
theie is no iesponse in 2 months, it is unlikely
to be effective. Monitoi foi signs of eaily stei-
oid atiophy.
· To¡íta| ta|tíneurín ín|í|íìors Taciolimus and
pimeciolimus aie effective in iepigmenting
vitiligo but only in sun-exposed aieas. They
aie iepoited to be most effective when com-
bined with UVB oi excimei lasei theiapy (see
below).
· To¡íta| ¡|oìot|emoì|era¡y Employs topical
8-methoxypsoialen (8-MOP) and UVA. This
pioceduie should be undeitaken foi small
macules only by expeiienced physicians and
well-infoimed patients. As with oial psoi-
alens, it may iequiie 15 tieatments to initiate
iesponse and 100 to finish.
· Sysìemít ¡|oìot|emoì|era¡y Foi moie wide-
spiead vitiligo, oial PUVA is moie piactical.
Oial PUVA may be done using sunlight
(in summei oi in aieas with yeai-iound
sunlight) and 5-methoxypsoialen (5-MOP)
(available in Euiope) oi with aitificial
UVA and eithei 5-MOP oi 8-MOP (Fig.
13-5). A iesponse to PUVA is signaled by
the appeaiance of tiny, usually folliculai
macules of pigmentation (Fig. 13-3). When
this occuis, it is a good piognostic sign
foi successful iepigmentation. Oial PUVA
photochemotheiapy with eithei 8-MOP oi
5-MOP is up to 85% effective in >70%
of patients with vitiligo of the head, neck,
uppei aims and legs, and tiunk. Howevei,
at least 1 yeai of tieatment is iequiied to
achieve this iesult. Distal hands and feet and
the °lip-tip" vaiiant of vitiligo aie pooily
iesponsive and, when piesent alone, aie not
usually woith tieating. Genital aieas should
be shielded and not tieated. Foi iisks of
PUVA theiapy see Section 3, °PUVA Theiapy
foi Psoiiasis."
· Narrow-|anJ UVB, J11 nm This is just as
effective as PUVA and does not iequiie
psoialens. It is the tieatment of choice in
childien <6 yeais of age.
· Excimei lasei (308 nm) This is effective but,
as foi PUVA, iepigmentation is also slow.
Pioduces best iesults in the face.
Mìoì¿raItìo¿
Minigiafting (autologous Thieisch giafts, suc-
tion blistei giafts, autologous minipunch giafts,
tiansplantation of cultuied autologous melano-
cytes) may be a useful technique foi iefiactoiy
and stable segmental vitiligo macules. PUVA may
be iequiied aftei the pioceduie to unify the coloi
between the giaft sites. The demonstiated occui-
ience of Koebneiization in donoi sites in geneial-
ized vitiligo iestiicts this pioceduie to those who
have limited cutaneous aieas at iisk foi vitiligo.
°Pebbling" of the giafted site may occui.
0epì¿meotatìoo
The objective of depigmentation is °one" skin
coloi in patients with extensive vitiligo oi in
those who have failed PUVA, who cannot use
PUVA, oi who ieject the PUVA option.
B|euchIng Bleaching of norma||y ¡ígmenìeJ
s|ín with monobenzylethei of hydioquinone
20% (MEH) cieam is a peimanent, iiieveis-
ible piocess. Since application of MEH may be
associated with satellite depigmentation, this
tieatment cannot be used selectively to bleach
ceitain aieas of noimal pigmentation, since
theie is a ieal likelihood that new and distant
white macules will develop ovei the months of
use. The success iate is >90%. The end-stage
coloi of depigmentation with MEH is chalk-
white, as in vitiligo macules. The patient who
may want bleaching with MEH is typically a
skin phototype IV to VI with extensive iepig-
mentation theiapy-iesistant vitiligo of the face
and hands with iesidual aieas of noimal (daik)
skin coloi who aie happy with a unifoim, albeit
white skin coloi on the exposed iegions. An
occasional patient may wish to take 30-60 mg
-caiotene pei day to impait an off-white coloi
to the vitiliginous skin.
All those who have bleached aie at iisk foi
sunbuin fiom acute solai iiiadiation.
No long-teim untowaid effects have been
iepoited fiom the use of MEH 20% cieam,
but note that the Je¡ígmenìaìíon at|íe·eJ ís
¡ermanenì .
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 341
A|||u|ºr deºc|||eº + ç|oup o| çeue||c +||e|+||ouº
o| ||e re|+u|u p|çreu| º,º|er ||+| +||ec| º||u,
|+|| |o|||c|eº, +ud e,eº.
|| p||uc|p+||, |u.o|.eº ||e º,u||eº|º o| re|+u|u |u
||eºe º||eº, |u| + uo|r+| uur|e| o| re|+uoc,|eº
+|e p|eºeu|.
A|||u|ºr c+u +||ec| ||e e,eº. ocu|+| +|||u|ºr
(0A), o| ||e e,eº +ud º||u. ocu|ocu|+ueouº
+|||u|ºr (0CA), o| ||e º||u +ud o||e| o|ç+u
º,º|erº, ||e C|' r+, +|ºo |e +||ec|ed |u ºore
|o|rº.
I|e c|+ºº|||c+||ou o| +|||u|ºr |º º|oWu |u
I+||e ¹!·¹.
0CA |º |, |+| ||e roº| corrou |o|r o| +|||u|ºr
+ud |º ||e ou|, |o|r d|ºcuººed |e|e.
AI8INISM |C|·9 . 210.2
°
|C|·¹0 . E10.!
£FI0£MI0I0C¥
C|assìIìcatìoo See Table 13-1.
Freva|eoce Estimated 1:20,000. OCA1 and
OCA2 account foi 40-50%.
Ioherìtaoce Most autosomal iecessive.
0CüI0CüIAN£0üS AI8INISM (0CA)
FICük£ 13-5 vìtì|ì¿o: therapy-ìoduced repì¿meotatìoo I||º 20·,e+|·o|d |ud|+u |er+|e |º |e|uç ||e+|ed
W||| p|o|oc|ero||e|+p, (|u\A). I|e|e |º º||ç|| e|,||er+ |u ||e .|||||ç|uouº r+cu|eº |u ||e e+||, p|+ºeº (|e||) o|
||e|+p, ||+| W||| |e |o||oWed |, |o|||cu|+| p|çreu|+||ou +º |u ||ç. ¹!·!, +||e| ¹ ,e+| o| ||e+|reu|, .|||||ço |+º cor·
p|e|e|, |ep|çreu|ed |u| ||e|e |º uoW |,pe|p|çreu|+||ou o| ||e |ueeº (||ç||). I||º, |oWe.e|, W||| |+de W||| ||re
+ud ||e co|o| o| ||e |ep|çreu|ed +|e+º W||| ||eud W||| ||+| o| ||e ºu||ouud|uç º||u.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 342
FAIh0C£N£SIS
The defect in melanin synthesis has been shown
to iesult fiom absence of the activity of the
enzyme tyiosinase. Tyiosinase is a coppei-
containing enzyme that catalyzes the oxidation
of tyiosine to dopa and the subsequent con-
veision of dopa to dopa-quinone. The muta-
tions in the tyiosinase gene iesponsible foi
deficient tyiosinase activity in seveial types of
albinism aie shown in Table 13-1.
CIINICAI MANIF£SIAII0N
0uratìoo Piesent at biith. Patients with albi-
nism avoid the sun because of iepeated sun-
buins and biight light because of pioblems
with vision; otheiwise, they live an essentially
noimal life.
Ceoera| Appearaoce °Poiing" (eyes half closed,
squinting) when in sunlight (Fig. 13-6).
Skìo Vaiied, depending on the type: °Snow"
white, cieamy white, light tan (Fig. 13-6 and
Table 13-1).
haìr White (tyiosinase-negative) (Fig. 13-6);
yellow, cieam, oi light biown (tyiosinase-
positive); ied; platinum.
£yes The eye changes aie the essential physical
finding that define the syndiome of OCA.
Nystagmus, a featuie always piesent, iesults
fiom hypoplasia of the fovea with ieduction of
visual acuity and alteiation in the foimation
of the optic neives; this misiouting of the
optic paths is also associated with an alteinat-
ing stiabismus and diminished steieoacuity.
The diagnostic featuies in the eye that identify
albinism aie theiefoie nystagmus and iiis tians-
lucency (Fig. 13-7), ieduction of visual acuity,
decieased ietinal pigment, foveal hypoplasia,
and stiabismus.
IA8I£ 13-1 C|assification of A|binism
6eoe
Type S0btypes Loc0s |oc|0des 0||o|ca| F|od|ogs
0CA¹ 0CA¹A íì| I,|oº|u+ºe·ueç+||.e 0CA w|||e |+|| +ud º||u, e,eº (p|u| +| |||||
||ue)
0CA¹B íì| \|u|r+| p|çreu| 0CA w|||e |o ue+|·uo|r+| º||u +ud |+||
p|çreu|+||ou
||+||uur 0CA
\e||oW 0CA \e||oW (p|eore|+u|u) |+||, ||ç|| |ed o|
||oWu |+||
Ierpe|+|u|e·ºeuº|||.e 0CA \+, |+.e ue+|·uo|r+| p|çreu| |u| uo| |u
+\|||+
Au|oºor+| |eceºº|.e 0CA (ºore)
0CA2 | I,|oº|u+ºe·poº|||.e 0CA \e||oW |+||, º||u 'c|e+r,¨ W|||e (A|||c+)
B|oWu 0CA ||ç|| ||oWu/|+u º||u (A|||c+)
0CA! íì||! Au|oºor+| |eceºº|.e 0CA (ºore)
ku|ouº 0CA ked +ud |ed·||oWu º||u +ud ||oWu e,eº
(A|||c+)
0CA+ !1í|
n|' ||' ne|r+uº|,·|ud|+| º,ud|ore '||u/|+|| +º |u 0CA¹A o| 0CA¹B o|
0CA2, ||eed|uç d|+||eº|º (|ue||o k|co)
Cn' |ì'í C|ed|+|·n|ç+º|| º,ud|ore '||.e| |+||/|,pop|çreu|+||ou/ºe||ouº
red|c+| p|o||erº
0A¹ 01! /·||u|ed 0A |o|r+| p|çreu|+||ou o| º||u +ud |+||
|0IE. 0CA, ocu|ocu|+ueouº +|||u|ºr, I\k, |,|oº|u+ºe, |, p|u| p|o|e|u, I\k|¹, |,|oº|u+ºe·|e|+|ed p|o|e|u ¹, 0A, ocu|+| +|||u|ºr, \AI|, rer||+ue·
+ººoc|+|ed ||+uºpo||e| p|o|e|u, |\'I, |,ºoºore ||+|||c||uç.
'0ukCE. \od|||ed ||or | B+|+do|+u e| +|, |u |\ ||eed|e|ç e| +|, (edº). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤-, o|| ed. |eW \o||, \cC|+W·
n|||, 200!.
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 343
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y LIght MIcrvscvpy Mel-
anocytes aie piesent in the skin and haii bulb
in all types of albinism. The dopa ieaction is
maikedly ieduced oi absent in the melanocytes
of the skin and haii, depending on the type
of albinism (tyiosinase-negative oi tyiosinase-
positive).
E|ectrvn MIcrvscvpy Melanosomes aie pie-
sent in melanocytes in all types of albinism, but
depending on the type of albinism, theie is a
ieduction of the melanization of melanosomes,
with many melanosomes being completely un-
melanized in tyiosinase-negative albinism.
Mo|ecu|ar Iestìo¿ Now available, and this
makes it possible to classify the specific gene
alteiation in vaiious types of albinism. Howevei,
it is not necessaiy foi diagnosis oi management
of the pioblem.
0IACN0SIS
White peisons with veiy faii skin (SPT I), blond
haii, and blue eyes may mimic albinos, but they
do not have eye changes (iiis tianslucency, nys-
tagmus). Some peisons with albinism who have
constitutive black oi biown skin coloi may have
a dilution of theii skin coloi fiom black to a light
FICük£ 13-T Irìs traos|uceocy wìth
a|bìoìsm |||º ||+uº|uceuc, |º + º|ue qu+
uou |u +|| |,peº o| ocu|ocu|+ueouº +|||·
u|ºr, e.eu |u ||oºe p+||eu|º |u W|or ||e
|||º |º ||oWu. I|e |||º |º |+|e|, p|u| e\cep|
|u |u|+u|º, +ud ||e d|+çuoº|º o| +|||u|ºr
depeudº ou ||e de|ec||ou o| |||º ||+uº|u·
ceuc,. I||º |º |eº| doue |u + d+|| |oor
W||| + ||+º|||ç|| po|u|ed +| ||e ºc|e|+.
FICük£ 13-6 0cu|ocutaoeous
a|bìoìsm w|||e º||u, W|||e e,e|+º|eº,
e,e||oWº, +ud ºc+|p |+||. I|e |||ºeº +ppe+|
||+uº|uceu|. nere p|çreu| ç|.eº ||e |+ce +
p|u||º| |ue.I|e|e |º ºqu|u||uç due |o p|o|o·
p|o||+ +ud u,º|+çruº.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 344
biown and have the capacity to tan; also, some
types may have biown iiides but still have iiis
tianslucency. Theiefoie, iiis tianslucency and
the piesence of othei eye findings in the fundus
aie the pathognomonic signs of albinism. The
haii and skin coloi may vaiy fiom noimal to
absent melanin, and the vaiious types aie listed
in Table 13-1. The special types of albinism aie
diagnosed on the basis of clinical piesentation
of the haii and skin pigmentation as well as
hematologic studies (Heimansky-Pudlak and
Chédiak-Higashi syndiome).
SICNIFICANC£
Albinism is an impoitant disease to iecognize
eaily in life in oidei to begin piophylactic
measuies to pievent deimatoheliosis and skin
cancei, i.e., piotective clothing, sunblocks, and
sun avoidance in peak peiiods duiing the day.
C0ükS£ AN0 Fk0CN0SIS
Albinos with tyiosinase-positive OCA foim
melanin pigment in the haii, skin, and eyes dui-
ing eaily life, the haii becoming cieam, yellow,
oi light biown, and the eye coloi changing fiom
light giay to blue, hazel, oi even biown.
Albinos living in cential Afiica who aie un-
piotected fiom the sun develop squamous cell
caicinomas eaily in life, and this significantly
shoitens theii life span; few suivive to the age
of 40 yeais because of metastasizing squamous
cell caicinoma. Deimatoheliosis and basal cell
caicinomas aie fiequent in albinos living in
tempeiate climates. Melanomas aie, cuiiously,
veiy iaie in albinos living in Afiica; when they
occui, they aie usually amelanotic. Melanocytic
nevi occui in albinism and may also be amelan-
otic but may be pigmented, depending on the
type of albinism.
MANAC£M£NI
Eveiy albino should be undei the caie of an
ophthalmologist foi vision pioblems and a dei-
matologist to detect solai keiatoses, skin canceis,
and deimatoheliosis. Daily application of topi-
cal, potent, bioad-spectium SPF >30 sunblocks,
including lip sunblocks. Avoidance of sun expo-
suie in the high solai intensity season. Use of
topical tietinoin foi deimatoheliosis and foi its
possible piophylactic effect against sun-induced
epithelial skin canceis. Tieatment of solai keia-
toses to pievent the development of squamous
cell caicinomas. Systemic -caiotene (30-60
mg thiice daily) impaits a moie noimal coloi
to the skin and may have some piotective effect
on the development of skin canceis, although
this has been pioved only in mice. It is helpful
foi albinos to belong to a national volunteei
gioup of albinos; in the United States it is called
the N ational O iganization foi À lbinism and
H ypomelanosis (NOAH). (Noah, the buildei of
the aik in the Old Testament, was alleged to be
an albino.) This gioup assists albinos in vaiious
ways, especially in dealing with vision piob-
lems: obtaining diivei`s license, etc.
£FI0£MI0I0C¥
A¿e oI 0oset Young adults.
Sex Females > males; about 10% of patients
with melasma aie men.
\e|+ºr+ (C|ee|. '+ ||+c| ºpo|¨) |º +u +cqu||ed
||ç||· o| d+||·||oWu |,pe|p|çreu|+||ou ||+|
occu|º |u ||e e\poºed +|e+º, roº| o||eu ou ||e
|+ce, +ud |eºu||º ||or e\poºu|e |o ºuu||ç||.
|| r+, |e +ººoc|+|ed W||| p|eçu+uc,, W||| |uçeº·
||ou o| cou||+cep||.e |o|roueº, o| poºº|||, W|||
ce||+|u red|c+||ouº ºuc| +º d|p|eu,||,d+u|o|u, o|
|| r+, |e |d|op+|||c.
',¤¤¤,¤º . C||o+ºr+ (C|ee|. '+ ç|eeu ºpo|¨ ),
r+º| o| p|eçu+uc,.
M£IASMA |C|·9 . 109.o9
°
|C|·¹0 . |3¹.¹
kace Melasma is moie appaient oi moie fie-
quent in peisons with biown oi black constitu-
tive skin coloi (peisons fiom Asia, the Middle
East, India, South Ameiica).
Iocìdeoce aod Frecìpìtatìo¿ Factors Veiy com-
mon, especially among peisons with constitutive
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 345
biown skin coloi who aie taking contiaceptive
iegimens and who live in sunny aieas. Pieg-
nancy causes melasma. Melasma has iecently
been appeaiing in menopausal women as a
iesult of iegimens foi pievention of osteopoio-
sis using a combination of estiogens anJ pio-
gesteione; melasma does not appeai in those
women who aie given estiogen ieplacement
tieatment but without piogesteione. Also in
patients on diphenylhydantoin. Sun ex¡osure
requíreJ.
FAIh0C£N£SIS
Unknown.
CIINICAI MANIF£SIAII0N
0uratìoo oI Iesìoos The pigmentation usually
evolves quite iapidly ovei weeks, paiticulaily
aftei exposuie to sunlight.
Skìo Iesìoos Maculai hypeipigmentation
of the face, the hue and intensity depending
laigely on the skin phototype of the patient
(Fig. 13-8). Light oi daik biown oi even black.
Coloi is usually unifoim but may be splotchy.
Most often symmetiic. Lesions have seiiated,
iiiegulai, and geogiaphic boideis. Two-thiids
on cential pait of the face: cheeks (Fig. 13-8),
foiehead, nose, uppei lip, and chin; a smallei
peicentage on the malai oi mandibulai aieas
of the face and occasionally the doisa of the
foieaims.
Wvvd Lump ErumInutIvn A maiked accen-
tuation of the hypeipigmented macules. T|ís
tonìrasì ís noì attenìuaìeJ ín ¡aìíenìs wíì| a
norma| |rown or ||at| s|ín .
0IFF£k£NIIAI 0IACN0SIS
Postinflammatoiy hypeimelanotic macules.
SICNIFICANC£
While this is a stiictly cosmetic pioblem, it
is veiy distuibing to both males and females,
especially peisons with biown skin coloi and
good tanning capacity.
C0ükS£ AN0 Fk0CN0SIS
Melasma may disappeai spontaneously ovei
a peiiod of months aftei deliveiy oi aftei
cessation of contiaceptive hoimones. Melasma
may oi may not ietuin with each subsequent
piegnancy.
FICük£ 13-8 Me|asma we||·der+|c+|ed, |,pe|p|çreu|ed r+cu|eº +|e ºeeu ou ||e c|ee|, uoºe, +ud uppe| ||p.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 346
FICM£NIAk¥ ChANC£S F0II0WINC INFIAMMAII0N 0F Ih£ SkIN
MANAC£M£NI
Iopìca| Commeicially available piepaiations
in the United States include: hydioquinone
3% solution and 4% cieam; azelaic acid 20%
cieam; and a combination of flucinolone
0.01%, hydioquinone 4%, and tietinoin 0.05%.
Hydioquinone 4% cieam can be compounded
with 0.05% tietinoin cieam oi glycolic acid by
the phaimacist.
UnJer no tírtumsìantes s|ou|J mono|en-
:y|eì|er o[ |yJroquínone or ì|e oì|er eì|ers o[
|¤º|·¤||c¤¤c|¤·, -¤·!-·¤c| ¤-|c¤·¤ |,¤-·¤·¸·
¤-¤|c|·¤¤ |º + r+jo| p|o||er |o| p+||eu|º W|||
º||u p|o|o|,peº |\, \, +ud \| (||çº. ¹!·9 +ud
¹!·¹0). I||º d|º||çu||uç p|çreu|+||ou c+u de.e|op
W||| +cue (||ç. ¹!·9), pºo||+º|º, ||c|eu p|+uuº (||ç.
¹!·¹0), +|op|c de|r+||||º, o| cou|+c| de|r+||||º
o| +||e| +u, |,pe o| ||+ur+ |o ||e º||u. || r+,
pe|º|º| |o| Wee|º |o rou||º |u| doeº |eºpoud |o
|op|c+| |,d|oqu|uoue, W||c| +cce|e|+|eº ||º d|º·
+ppe+|+uce. |eº|ouº +|e c|+|+c|e||º||c+||, ||r||ed
|o ||e º||e o| ||e p|eced|uç |u||+rr+||ou +ud
|+.e |ud|º||uc|, |e+||e|ed |o|de|º.
'ore d|uç e|up||ouº r+, |e +ººoc|+|ed W|||
de|r+| re|+u|u |,pe|p|çreu|+||ou (||ç. ¹!·¹¹),
W||c| r+, +|ºo |e +ººoc|+|ed W||| ||c|eu p|+uuº
+ud cu|+ueouº |upuº e|,||er+|oºuº. I||º de|r+|
|,pe|p|çreu|+||ou r+, |e pe|º|º|eu|, +ud ||e|e
|º uo ||e+|reu|.
|·-|| ¤-|c¤¤º·º (re|+uode|r+||||º |o\|c+) |º +
|e||cu|+|, cou||ueu| ||+c| |o ||oWu·.|o|e| p|çreu·
|+||ou o| ||e |+ce +ud uec| (||ç. ¹!·¹2). || r+,
|e + |eºu|| o| cou|+c| ºeuº|||.||, o| p|o|ocou|+c|
ºeuº|||.||, |e|+|ed |o c|er|c+|º, p+|||cu|+||, ||+·
ç|+uce |u coºre||cº.
|¤· |,¤-·¤-|c¤¤º·º !±- |¤ ¤|¤|¤|¤··: ·-c:|·¤¤º
|uduced |, pºo|+|euº (Be||oque de|r+||||º), ºee
'ec||ou ¹0, +ud |o| ¤¤¤·¤-|c¤·¤·|cº-! |,¤-·¤·¸·
¤-¤|c|·¤¤ due |o d|uçº, ºee 'ec||ou 22.
|yJroquínone (monomeì|y|- or monoeì|y|-) |e
useJ ín ì|e ìreaìmenì o[ me|asma |etause ì|ese
Jrugs tan |eaJ ìo a ¡ermanenì |oss o[ me|anotyìes
wíì| ì|e Je·e|o¡menì o[ a Jís[íguríng s¡oììy |eu-
|oJerma .
Freveotìoo It is essential that the patient use,
eveiy moining, an o¡aque sunblock containing
titanium dioxide and/oi zinc oxide; the action
spectium of pigment daikening extends into
the visible iange, and even the potent tians-
paient sunscieens (with high SPF) aie com-
pletely ineffective in blocking visible iadiation.
h¥F£kFICM£NIAII0N |C|·9 . 109.0
°
|C|·¹0 . |3¹.9
FICük£ 13-10 FostìoI|ammatory hyperpì¿meotatìoo (Ac|oºº |+c|uç p+çe) I||º r+, |o||oW + d|uç
e|up||ou, pºo||+º|º, o| ||c|eu p|+uuº, eºpec|+||, |u º||u p|o|o|,peº \ +ud \|, +º W+º ||e c+ºe |u |||º r|dd|e·+çe
E+º| |ud|+u r+|e. |oº||u||+rr+|o|, |,pe|p|çreu|+||ou |º + r+jo| p|o||er |u ,ouuç |er+|eº W||| º||u p|o|o|,peº
\ +ud \|.
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 34T
FICük£ 13-9 hyperme|aoosìs wìth acoe â. I||º coud|||ou |º + r+jo| corp|+|u| o| |||º ¹3·,e+|·o|d º||u
p|o|o|,pe ||| ç|||. I|e +cue |º uo| ||e p|o||er uoW, || |º ||e d|º||çu||uç |,pe|re|+uoº|º. I||º |,pe|p|çreu|+||ou
c+u |e r+||ed|, |educed W||| |op|c+| |,d|oqu|uoue (!°) c|e+r o| ºo|u||ou, |eº| cor||ued W||| ||e||uo|u, +pp||ed
d+||,. |u||uç ||e dep|çreu|+||ou, ||e p+||eu| ruº| uºe +u op+que ºuu||oc| d+||, |o p|e.eu| ||e p|çreu| d+||eu|uç
||+| occu|º W||| d+||, ºuu e\poºu|e. 8. |u |||º !0·,e+|·o|d |+||º|+u| Wor+u |,pe|re|+uoº|º due |o +cue, cor||ued
W||| re|+ºr+ +ud |,pop|çreu|ed +cue ºc+|º, W+º couº|de|ed + coºre||c d|º+º|e|, uo| ou|, |, ||e p+||eu| |u| +|ºo
|e| |uº|+ud. '|e W+º ºucceºº|u||, ||e+|ed W||| !° |,d|oqu|uoue |uco|po|+|ed |u|o + 0.05° ||e||uo|u c|e+r.
â 8
FICük£ 13-10
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 348
FICük£ 13-11 FostìoI|ammatory derma| hyperpì¿meotatìoo I||º |º º|oWu ou ||e |+ud o| + º||u p|o·
|o|,pe |\ A|||c+u Wor+u |o||oW|uç + ||\ed d|uç e|up||ou.
FICük£ 13-12 Me|aoodermatìtìs toxìca â. A |e||cu|+| cou||ueu| p|çreu|+||ou ou ||e |+ce +ud uec| o| + +2·
,e+|·o|d |er+|e c|er|º| W|o Wo||ed |o| + coºre||c |uduº||, +ud |+d +pp||ed, o.e| ,e+|º, roº| o| ||e ºceu|ed p|od·
uc|º º|e W+º |u.o|.ed |u p|oduc|uç |o |e| oWu º||u. '|uce º|e ||.ed |u + ºuuu, c||r+|e |||º |uc|e+ºeº ||e ºuºp|c|ou o| +
c||ou|c p|o|ocou|+c| ºeuº|||.||,. 8. |u |||º |ud|+u Wor+u ||e ro|||ed |,pe|p|çreu|+||ou |+º co+|eºced |o d+|| ||oWu
ro|||ed |,pe|p|çreu|+||ou o| ||e c|ee|º. |o| p|o|eºº|ou+| |e+ºouº |||º p+||eu| |+d +|ºo e\ceºº|.e|, uºed coºre||cº.
8 â
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 349
|oº||u||+rr+|o|, |,pore|+uoº|º |º +|W+,º |e|+|ed
|o |oºº o| re|+u|u. || |º + ºpec|+| |e+|u|e o| p||,||+·
º|º .e|º|co|o| (||ç. ¹!·¹!, ºee +|ºo 'ec||ou 25), |u
W||c| ||e |,pop|çreu|+||ou r+, +|ºo |er+|u |o|
Wee|º +||e| ||e +c||.e |u|ec||ou |+º d|º+ppe+|ed.
n,pore|+uoº|º |º uo| uucorrou|, ºeeu |u +|op|c
de|r+||||º, pºo||+º|º (||ç. ¹!·¹+), çu||+|e p+|+pºo·
||+º|º, +ud p||,||+º|º ||c|euo|deº c||ou|c+.
|| r+, +|ºo |e p|eºeu| |u cu|+ueouº |upuº
e|,||er+|oºuº (||ç. ¹!·¹5), +|opec|+ ruc|uoº+,
r,coº|º |uuço|deº, ||c|eu º|||+|uº, ºe|o|||e|c
de|r+||||º, +ud |ep|oº,.
n,pore|+uoº|º r+, |o||oW de|r+||+º|ou +ud
c|er|c+| pee|º, |u ||eºe coud|||ouº ||e|e |º
+ '||+uº|e| ||oc|,¨ |u W||c| re|+uoºoreº +|e
p|eºeu| |u re|+uoc,|eº |u| +|e uo| ||+uº|e||ed
|o |e|+||uoc,|eº, |eºu|||uç |u |,pore|+uoº|º. I|e
|eº|ouº +|e uºu+||, uo| c|+|| W|||e, +º |u .|||||ço,
|u| 'o||¨ W|||e +ud |+.e |ud|ºc|e|e r+|ç|uº.
A corrou |,pe o| |,pop|çreu|+||ou |º +ººoc|·
+|ed W||| ¤·|,··cº·º c||c (||ç. ¹!·¹o). I||º |º +
r+cu|+| |,pop|çreu|+||ou roº||, ou ||e |+ce o|
c|||d|eu, o||·W|||e W||| + poWde|, ºc+|e. ke|+||.e|,
|ud|º||uc| r+|ç|uº uude| wood ||ç|| +ud ºc+|·
|uç d|º||uçu|º| |||º ec/er+|ouº de|r+||||º ||or
.|||||ço. || |º ºe||·||r||ed.
n,pore|+uoº|º uo| uucorrou|, |o||oWº |u||+·
|eº|ou+| ç|ucoco|||co|d |ujec||ouº, |u| W|eu ||e
|ujec||ouº +|e º|opped, + uo|r+| p|çreu|+||ou
de.e|opº |u ||e +|e+º.
|epeud|uç ou ||e +ººoc|+|ed d|ºo|de|, poº||u·
||+rr+|o|, |,pore|+uoº|º r+, |eºpoud |o o|+|
|u\A p|o|oc|ero||e|+p,.
FICük£ 13-13 Fìtyrìasìs versìco|or â. n,pop|çreu|ed, º|+|p|, r+|ç|u+|ed, ºc+||uç r+cu|eº ou ||e |+c|
o| +u |ud|.|du+| W||| º||u p|o|o|,pe |||. Ceu||e +||+º|ou o| ||e ºu||+ce W||| +cceu|u+|e ||e ºc+||uç. I||º |,pe o|
|,pore|+uoº|º c+u |er+|u |ouç +||e| ||e e|up||ou |+º |eeu ||e+|ed +ud ||e p||r+|, p|oceºº |º |eºo|.ed. 8.
|||,||+º|º .e|º|co|o| |u A|||c+u º||u. |eº|ouº +|e pe|||o|||cu|+| ou ||e c|eº| +ud co+|eºce |o |+|çe cou||ueu| p+|c|eº
ou ||e uec| W|e|e ||e ||ue ºc+||uç c+u |eº| |e ºeeu.
â 8
h¥F0FICM£NIAII0N |C|·9 . 109.0
°
|C|·¹0 . |3¹.9
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 350
FICük£ 13-14 FostìoI|ammatory hypome|aoosìs (psorìasìs) I|e |,pore|+uo||c |eº|ouº co||eºpoud
e\+c||, |o ||e +u|ecedeu| e|up||ou. I|e|e |º ºore |eº|du+| pºo||+º|º W||||u ||e |eº|ouº.
S£CII0N 13 ||C\E|IAk\ ||'0k|Ek' 351
FICük£ 13-15 FostìoI|ammatory hypopì¿meotatìoo |,¤¤¤·¸¤-¤|c|·¤¤ |u + !!·,e+|·o|d \|e|u+reºe
|er+|e. I|e p+||eu| |+d |+d c||ou|c cu|+ueouº |upuº e|,||er+|oºuº. keº|du+| |u||+rr+||ou o| |upuº |º º|||| ºeeu
ou ||e uppe| ||p.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 352
FICük£ 13-16 Fìtyrìasìs a|ba A corrou d|º||çu||uç |,pore|+uoº|º, W||c|, +º ||e u+re |ud|c+|eº, |º +
W|||e +|e+ (+||+) W||| .e|, r||d ºc+||uç (p||,||+º|º). || |º o|ºe|.ed |u + |+|çe uur|e| o| c|||d|eu |u ||e ºurre| |u
|erpe|+|e c||r+|eº. || |º roº||, + coºre||c p|o||er |u pe|ºouº W||| ||oWu o| ||+c| º||u +ud corrou|, occu|º ou
||e |+ce, +º |u |||º c|||d. Arouç 200 p+||eu|º W||| p||,||+º|º +||+, 90° |+uçed ||or o-¹2 ,e+|º o| +çe. |u ,ouuç
+du||º, |A qu||e o||eu occu|º ou ||e +|rº +ud ||uu|.
| A k I | |
DEkMAIOLOCY AND
INIEkNAL MEDICINE
354
S E C I | 0 N 1 4
Ih£ SkIN IN IMMüN£,
AüI0IMMüN£, AN0
kh£üMAIIC 0IS0k0£kS
Ar,|o|doº|º |º +u e\||+ce||u|+| depoº|||ou |u .+||·
ouº ||ººueº o| +r,|o|d |||||| p|o|e|uº +ud o| +
p|o|e|u c+||ed c¤,|¤·! | :¤¤¤¤¤-¤| (A|), ||e
|deu||c+| corpoueu| o| A| |º p|eºeu| |u ||e ºe|ur
+ud |º c+||ed '1| . I|eºe +r,|o|d depoº||º c+u
+||ec| uo|r+| |od, |uuc||ou.
',º|-¤·: 1| c¤,|¤·!¤º·º , +|ºo |uoWu +º ¤··¤c·,
c¤,|¤·!¤º·º , occu|º |u p+||eu|º W||| B ce|| o|
p|+ºr+ ce|| d,ºc|+º|+º +ud ru|||p|e r,e|or+ |u
W|or ||+çreu|º o| rouoc|ou+| |rruuoç|o|u||u
||ç|| c|+|uº |o|r +r,|o|d ||||||º.
C||u|c+| |e+|u|eº o| A| |uc|ude + cor||u+||ou o|
r+c|oç|oºº|+ +ud c+|d|+c, |eu+|, |ep+||c, +ud
ç+º||o|u|eº||u+| (C|) |u.o|.ereu|, +º We|| +º c+|p+|
|uuue| º,ud|ore +ud º|·¤ |-º·¤¤º . I|eºe occu|
|u !0° o| p+||eu|º, +ud º|uce ||e, occu| e+||,
|u ||e d|ºe+ºe, ||e, +|e +u |rpo||+u| c|ue |o ||e
d|+çuoº|º.
',º|-¤·: 11 c¤,|¤·!¤º·º (|e+c||.e) occu|º |u
p+||eu|º +||e| c||ou|c |u||+rr+|o|, d|ºe+ºe, |u
W|or ||e |||||| p|o|e|u |º de||.ed ||or ||e c||·
cu|+||uç +cu|e·p|+ºe ||pop|o|e|u |uoWu +º º-·±¤
c¤,|¤·! 1 .
I|e|e +|e |eW o| uo c|+|+c|e||º||c º||u |eº|ouº |u
AA +r,|o|doº|º, W||c| uºu+||, +||ec|º ||e ||.e|,
ºp|eeu, ||due,º, +ud +d|eu+|º.
|u +dd|||ou, º||u r+u||eº|+||ouº r+, +|ºo |e
+ººoc|+|ed W||| + uur|e| o| (|+|e) |e|edo|+r|||+|
º,ud|oreº.
|¤:c|·c-! :±|c¤-¤±º c¤,|¤·!¤º·º |º uo| uucor·
rou, p|eºeu|º W||| |,p|c+| cu|+ueouº r+u||eº|+·
||ouº, +ud |+º uo º,º|er|c |u.o|.ereu|.
S¥SI£MIC AM¥I0I00SIS |C|·9 . 211.!
°
|C|·¹0 . E35.!
CIINICAI MANIF£SIAII0N
Skìo Iesìoos Smooth, waxy papules (Fig. 14-1),
but also nodules on the face, especially aiound
the eyes (Fig. 14-2) and elsewheie. Puipuia
following tiauma, °pinch" puipuia in waxy pa-
pules (Fig. 14-2) sometimes also involving laige
suiface aieas without nodulai involvement.
Piedilection sites aie aiound the eyes, cential
face, extiemities, body folds, axillae, umbilicus,
anogenital aiea. Naí| t|anges : Similai to lichen
k+|e
0ccu|º |u r+u, |u| uo| +|| p+||eu|º W||| ru|·
||p|e r,e|or+ +ud B ce|| +ud p|+ºr+ ce||
d,ºc|+º|+º.
keº|||c||.e c+|d|or,op+||,, |eu+| |uuc||ou |r·
p+||reu|, C| |u.o|.ereu| W||| r+|+|ºo|p||ou,
ueu|op+||,.
||oçuoº|º poo|
S¥SI£MIC AI AM¥I0I00SIS |C|·9. 211.!
°
|C|·¹0 . E35
planus (see Section 33). Matrog|ossía : diffusely
enlaiged and fiim, °woody" (Fig. 14-3).
Systemìc MaoìIestatìoos Include fatigue, weak-
ness, anoiexia, weight loss, malaise, dyspnea;
symptoms ielated to hepatic, ienal, and GI
involvement; paiesthesia ielated to caipal tunnel
syndiome, neuiopathy.
Ceoera| £xamìoatìoo Kidney-nephiosis; neiv-
ous system-peiipheial neuiopathy, caipal tunnel
syndiome; caidiovasculai-paitial heait block,
congestive heait failuie; hepatic-hepatomegaly;
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 355
GI-diaiihea, sometimes hemoiihagic, malab-
soiption; lymphadenopathy.
IA80kAI0k¥ £XAMINAII0NS
May ieveal thiombocytosis >500,000/µL.
Pioteinuiia and incieased seium cieatinine;
hypeicalcemia. Incieased IgG. Monoclonal pio-
tein in two-thiids of patients with piimaiy oi
myeloma-associated amyloidosis. Bone mai-
iow: myeloma.
0ermatopatho|o¿y Shows accumulation of
faintly eosinophilic masses of amyloid in the
papillaiy body neai the epideimis, in the papil-
laiy and ieticulai deimis, in sweat glands, aiound
and within blood vessel walls. Use thioflavin oi
congo ied and examine the sections foi an ap-
ple-gieen biiefiingence with a polaiization mi-
cioscope. Immunohistochemistiy to assess the
piopoition of kappa and lambda light chains.
0IACN0SIS
Made by the combination of puipuiic skin
lesions (Fig. 14-2), waxy papules (Fig. 14-1),
macioglossia (Fig. 14-3), caipal tunnel syn-
diome, and caidiac symptoms. A tissue di-
agnosis can be made fiom the skin biopsy.
Scintigiaphy aftei injection of
123
I-labeled SAP
will ieveal the extent of the involvement and
can seive as a guide foi tieatment, which is that
of the undeilying disease.
FICük£ 14-1 Systemìc AI
amy|oìdosìs w+\, p+pu|eº ou ||e
||uu| o| + 53·,e+|·o|d r+|e p+||eu|
W||| r,e|or+.
FICük£ 14-2 Systemìc AI amy-
|oìdosìs: "pìoch purpura" I|e
|oproº| p+pu|e |º ,e||oW|º| +ud
uou|ero|||+ç|c, ||e |oWe| po|||ou |º
|ero|||+ç|c. 'o·c+||ed p|uc| pu|pu|+
o| ||e uppe| e,e||d c+u +ppe+| |u
+r,|o|d uodu|eº +||e| p|uc||uç o| |u|·
||uç ||e e,e||d.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 356
A |e+c||.e |,pe o| +r,|o|doº|º.
0ccu|º |u +u, d|ºo|de| +ººoc|+|ed W||| + ºuº|+|ued
+cu|e·p|+ºe |eºpouºe.
o0° |+.e |u||+rr+|o|, +|||||||º. I|e |eº| o||e|
c||ou|c |u||+rr+|o|, |u|ec||.e o| ueop|+º||c d|º·
o|de|º.
Ar,|o|d ||||||º +|e de||.ed ||or c|e+.+çe
||+çreu|º o| ||e c||cu|+||uç +cu|e·p|+ºe |e+c|+u|
ºe|ur +r,|o|d A p|o|e|u.
||eºeu|º W||| p|o|e|uu||+ |o||oWed |, p|oç|eºº|.e
|eu+| d,º|uuc||ou, uep||o||c º,ud|ore.
I|e|e +|e uo c|+|+c|e||º||c º||u |eº|ouº |u AA
+r,|o|doº|º.
S¥SI£MIC AA AM¥I0I00SIS |C|·9. 211.!
°
|C|·¹0 . E35
I||ee .+||e||eº o| |oc+||/ed +r,|o|doº|º ||+| +|e
uu|e|+|ed |o ||e º,º|er|c +r,|o|doºeº.
|¤!±|c· c¤,|¤·!¤º·º . º|uç|e o| ru|||p|e, ºroo||,
uodu|+| |eº|ouº W||| o| W|||ou| pu|pu|+ ou ||r|º,
|+ce, o| ||uu| (||ç. ¹+·+1).
|·:|-¤¤·! c¤,|¤·!¤º·º. d|ºc|e|e, .e|, p|u||||c,
||oWu|º|·|ed p+pu|eº ou ||e |eçº (||ç. ¹+·+3).
!c:±|c· c¤,|¤·!¤º·º . p|u||||c, ç|+,·||oWu, |e||cu·
|+|ed r+cu|+| |eº|ouº occu|||uç p||uc|p+||, ou ||e
uppe| |+c| (||ç. ¹+·5), ||e |eº|ouº o||eu |+.e +
d|º||uc||.e '||pp|e¨ p+||e|u.
|u ||c|euo|d +ud r+cu|+| +r,|o|doº|º ||e +r,|o|d
||||||º |u º||u +|e |e|+||u·de||.ed. A|||ouç| ||eºe
|||ee |oc+||/ed |o|rº o| +r,|o|doº|º +|e cou||ued
|o ||e º||u +ud uu|e|+|ed |o º,º|er|c d|ºe+ºe, ||e
º||u |eº|ouº o| uodu|+| +r,|o|doº|º +|e |deu||c+|
|o ||oºe ||+| occu| |u A|, |u W||c| +r,|o|d ||||||º
de||.e ||or |rruuoç|o|u||u ||ç|| c|+|u ||+ç·
reu|º.
I0CAIII£0 CüIAN£0üS AM¥I0I00SIS
FICük£ 14-3 Systemìc AA amy|oìdosìs: macro¿|ossìa \+ºº|.e |u|||||+||ou o| ||e |ouçue W||| +r,|o|d
|+º c+uºed |rreuºe eu|+|çereu|, ||e |ouçue c+uuo| |e |e||+c|ed corp|e|e|, |u|o ||e rou|| |ec+uºe o| ||º º|/e.
(Cou||eº, o| E.+u C+|||uº, \|.)
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 35T
FICük£ 14-4 Ioca|ìted cutaoeous amy|oìdosìs â. |odu|+|. IWo p|+que|||e uodu|eº, W+\,, ,e||oW|º|·
o|+uçe W||| |ero|||+çe. 8. ||c|euo|d +r,|o|doº|º. C|ouped cou||ueu| ºc+|, p+pu|eº o| ||.|d, .|o|+ceouº co|o|.
I||º |º + pu|e|, cu|+ueouº d|ºe+ºe.
FICük£ 14-5 Macu|ar amy|oìdosìs C|+,·||oWu, |e||cu|+|ed p|çreu|+||ou ou ||e |+c| o| + 52·,e+|·o|d
A|+||+u r+|e.
â 8
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 358
£FI0£MI0I0C¥ AN0 £II0I0C¥
Iocìdeoce 15-23% of the population may
have had this condition duiing theii lifetime.
Chionic uiticaiia is likely to be piesent at some
time in about 25% of patients with uiticaiia.
£tìo|o¿y Uiticaiia/angioedema is not a disease
but a cutaneous ieaction pattein. Foi classifica-
tion and etiology, see Table 14-1.
CIINICAI I¥F£S
Acute ürtìcarìa Acute onset and iecuiiing
ovei <30 days. Usually laige wheals often as-
sociated with angioedema (Figs. 14-7 and 14-
8); often IgE-dependent with atopic diathesis;
ielated to foods, paiasites, and penicillin. Also,
complement-mediated in seium sickness-like
ieactions (whole blood, immunoglobulins,
penicillin). Often accompanied by angioedema.
Common. (See also °Diug-Induced Acute Uiti-
caiia," Section 22.)
Chrooìc ürtìcarìa Recuiiing ovei <30 days.
Small and laige wheals (Fig. 14-9). Raiely IgE-
dependent but often due to anti-Fc R autoanti-
bodies; etiology unknown in 80% and theiefoie
consideied idiopathic. Intoleiance to salicylates,
benzoates. Common. Chionic uiticaiia affects
adults piedominantly and is appioximately twice
as common in women as in men. Up to 40% of
patients with chionic uiticaiia of >6 months`
duiation still have uiticaiia 10 yeais latei.
Symptoms Prurítus In angioedema of palms
and soles pain. Angioedema of tongue, phai-
ynx inteifeies with speech, food intake, and
bieathing. Angioedema of laiynx may lead to
asphyxia.
u|||c+||+ |º corpoºed o| W|e+|º (||+uº|eu| eder+·
|ouº p+pu|eº +ud p|+queº, uºu+||, p|u||||c +ud due
|o eder+ o| ||e p+p|||+|, |od,) (||çº. ¹+·o +ud
¹+·1). I|e W|e+|º +|e ºupe|||c|+|, We|| de||ued.
Auç|oeder+ |º + |+|çe| eder+|ouº +|e+ ||+|
|u.o|.eº ||e de|r|º c¤! ºu|cu|+ueouº ||ººue
(||ç. ¹+·3) +ud |º deep +ud ||| de||ued. u|||c+||+
+ud +uç|oeder+ +|e ||uº ||e º+re eder+|ouº
p|oceºº |u| |u.o|.|uç d|||e|eu| |e.e|º o| ||e cu|+·
ueouº .+ºcu|+| p|e\uº. p+p|||+|, +ud deep.
|C|·9. 211.o
°
|C|·¹0 . |3+.¹
u|||c+||+ +ud/o| +uç|oeder+ r+, |e +cu|e |ecu|·
|eu| o| c||ou|c |ecu||eu|.
0||e| |o|rº o| u|||c+||+/+uç|oeder+ +|e |ecoç·
u|/ed. |
ç
E· +ud |
ç
E |ecep|o|-depeudeu|, p|,º|c+|,
cou|+c|, r+º| ce|| deç|+uu|+||ou-|e|+|ed, +ud
|d|op+|||c.
|u +dd|||ou, +uç|oeder+/u|||c+||+ c+u |e red|·
+|ed |, ||+d,||u|u, ||e corp|ereu| º,º|er, +ud
o||e| e||ec|o| rec|+u|ºrº.
u|||c+||+| .+ºcu||||º |º + ºpec|+| |o|r o| cu|+ueouº
uec|o||/|uç .euu||||º (ºee p+çe +01).
I|e|e +|e ºore º,ud|oreº W||| +uç|oeder+ |u
W||c| u|||c+||+| W|e+|º +|e |+|e|, p|eºeu| (e.ç.,
|e|ed||+|, +uç|oeder+).
ükIICAkIA AN0 ANCI0£0£MA |C|·9 . 103.0
°
|C|·¹0 . |50
CIINICAI MANIF£SIAII0N
Skìo Iesìoos Shaiply defined w|ea|s (Fig. 14-6),
small (<1 cm) to laige (>8 cm), eiythematous
oi white with an eiythematous iim, iound,
oval, aciifoim, annulai, seipiginous (Figs. 14-7
and 14-9), due to confluence and iesolution in
one aiea and piogiession in anothei (Fig. 14-7).
Lesions aie piuiitic and tiansient.
ÀngíoeJema - skin-coloied, tiansient en-
laigement of poition of face (eyelids, lips,
tongue) (Figs. 14-8 and 22-7À, B), extiemity, oi
othei sites due to subcutaneous edema.
DIstrIhutIvn Usually iegional oi geneialized.
Localized in solai, piessuie, vibiation, cold
uiticaiia/angioedema and confined to the site
of the tiiggei mechanism (see below).
SF£CIAI F£AIük£S[AS k£IAI£0
I0 FAIh0C£N£SIS
Immuoo|o¿ìc ürtìcarìa 1gE MedIuted
Lesions in acute IgE-mediated uiticaiia
iesult fiom antigen-induced ielease of bio-
logically active molecules fiom mast cells oi
basophilic leukocytes sensitized with specific
IgE antibodies (type I anaphylactic hypeisen-
sitivity). Released mediatois inciease venulai
peimeability and modulate the ielease of bio-
logically active molecules fiom othei cell types.
Often with atopic backgiound. Antigens: food
(milk, eggs, wheat, shellfish, nuts), theiapeutic
agents, diugs (penicillin) (see also °Diug-In-
duced Uiticaiia," Section 22), helminths. Most
often acute (Figs. 14-8 and 22-6).
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 359
IA8I£ 14-1 Etio|o¿y and C|assification of Urticaria/An¿ioedema
|rruuo|oç|c u|||c+||+ due |o r+º| ce||-|e|e+º|uç +çeu|º, pºeudo+||e|çeuº, ACE |u|||||o|º
|çE·red|+|ed u|||c+||+ |d|op+|||c u|||c+||+
Corp|ereu|·red|+|ed u|||c+||+ |ou|rruue cou|+c| u|||c+||+
Au|o|rruue u|||c+||+ u|||c+||+ +ººoc|+|ed W||| .+ºcu|+|/couuec||.e ||ººue +u|o|rruue d|ºe+ºe
|rruue cou|+c| u|||c+||+ ||º||uc| +uç|oeder+ (± u|||c+||+) º,ud|oreº
||,º|c+| ne|ed||+|, +uç|oeder+
|e|roç|+p||ºr Auç|oeder+·u|||c+||+·eoº|uop||||+ º,ud|ore
Co|d u|||c+||+
'o|+| u|||c+||+
C|o||ue|ç|c u|||c+||+
||eººu|e +uç|oeder+
\|||+|o|, +uç|oeder+
|0IE. ACE, +uç|o|euº|u·cou.e|||uç eu/,re.
FICük£ 14-6 ürtìcarìa w|e+|º W||| W|||e·|o·||ç||·p|u| co|o| |u ||e |+ce |u + c|oºe·up .|eW. I|eºe +|e ||e
c|+ºº|c |eº|ouº o| u|||c+||+. || |º c|+|+c|e||º||c ||+| ||e, +|e ||+uº|eu| +ud ||ç||, p|u||||c.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 360
Cvmp|ement MedIuted By way of immune
complexes activating complement and ieleasing
anaphylatoxins that induce mast cell degianula-
tion. Seium sickness, administiation of whole
blood, immunoglobulins. Acute.
AutvImmune Common, chionic. Autoanti-
bodies against Fc RI and/oi IgE. Positive au-
tologous seium skin test. Clinically, patients
with these autoantibodies (up to 40% of
patients with chionic uiticaiia) aie indistin-
guishable fiom those without them (Fig. 14-9).
These autoantibodies may explain why plas-
mapheiesis, intiavenous immunoglobulins, and
cyclospoiine induce iemission of disease activ-
ity in these patients.
1mmunv|vgIc Cvntuct UrtIcurIu Usually in
childien with atopic deimatitis sensitized to
enviionmental alleigens (giass, animals) oi
individuals sensitized to weaiing latex iubbei
gloves; can be accompanied by anaphylaxis.
Fhysìca| ürtìcarìas DermvgruphIsm Lineai
uiticaiial lesions occui aftei stioking oi
sciatching the skin; they itch and fade in 30
min (Fig. 14-10). 4.2% of the noimal popula-
tion have it; symptomatic deimogiaphism is a
nuisance.
Cv|d UrtIcurIu Usually in childien oi young
adults; uiticaiial lesions confined to sites
exposed to cold occuiiing within minutes aftei
iewaiming. °Ice cube" test (application of an
ice cube foi a few minutes to skin) establishes
diagnosis.
Sv|ur UrtIcurIu Uiticaiia aftei solai exposuie.
Action spectium fiom 290-500 nm; whealing
lasts foi <1 h, may be accompanied by syncope;
histamine is one of the mediatois (see Section
10 and Fig. 10-10).
Chv|InergIc UrtIcurIu Exeicise to the point of
sweating piovokes typical small, papulai, highly
piuiitic uiticaiial lesions (Fig. 14-11). May be
accompanied by wheezing.
AquugenIc UrtIcurIu Veiy iaie. Contact with
watei of any tempeiatuie induces eiuption
similai to cholineigic uiticaiia.
Pressure AngIvedemu Eiythematous swelling
induced by sustained piessuie (buttock swelling
when seated, hand swelling aftei hammeiing,
foot swelling aftei walking). Delayed (30 min
to 12 h). Painful, may peisist foi seveial days,
and inteifeies with quality of life. No laboiatoiy
abnoimalities; fevei may occui. Uiticaiia may
occui in addition to angioedema.
FICük£ 14-T Acute urtìcarìa 'r+|| +ud |+|çe W|e+|º W||| e|,||er+|ouº |o|de|º +ud + ||ç||e| co|o| ceu·
||+||,. we||·de||ued. I|e |eº|ou ou ||e |e|| uppe| +|r |º |||·de||ued +| ||º |oWe| |o|de| W|e|e || |º |eç|eºº|uç.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 361
FICük£ 14-8 Acute urtìcarìa aod ao¿ìoedema |o|e ||+| ||e|e +|e |o|| ºupe|||c|+| W|e+|º +ud deep,
d|||uºe eder+. 0ccu||ed +||e| ||e p+||eu| |+d e+|eu º|e||||º|. ne |+d º|r||+| ep|ºodeº p|e.|ouº|, |u| ue.e| cou·
º|de|ed ºe+|ood +º ||e c+uºe.
FICük£ 14-9 Chrooìc urtìcarìa C||ou|c
u|||c+||+ o| 5·,e+| du|+||ou |u +u o||e|W|ºe
|e+|||, !5·,e+|·o|d |er+|e. E|up||ouº occu|
ou +u +|roº| d+||, |+º|º +ud, +º ||e, +|e
||ç||, p|u||||c, ç|e+||, |rp+|| ||e p+||eu|'º
qu+|||, o| |||e. A|||ouç| ºupp|eººed |,
+u||||º|+r|ueº, ||e|e |º +u |rred|+|e |ecu|·
|euce +||e| ||e+|reu| |º º|opped. kepe+|ed
|+|o|+|o|, +ud c||u|c+| e\+r|u+||ouº |+.e uo|
|e.e+|ed +u +pp+|eu| c+uºe.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 362
VIhrutIvn AngIvedemu May be familial
(autosomal dominant) oi spoiadic. Raie. It
is believed to iesult fiom histamine ielease
fiom mast cells caused by a °vibiating" stimu-
lus-iubbing a towel acioss the back pioduces
lesions, but diiect piessuie (without move-
ments) does not.
ürtìcarìa 0ue to Mast Ce||-ke|easìo¿ A¿eots aod
Fseudoa||er¿eos aod Chrooìc Idìopathìc ürtìcarìa
Uiticaiia/angioedema and even anaphylaxis-
like symptoms may occui with iadiocontiast
media and as a consequence of intoleiance
to salicylates, food pieseivatives and additives
(e.g., benzoic acid and sodium benzoate), as
well as seveial azo dyes, including taitiazine and
sunset yellow (pseudoalleigens) (Fig. 14-9); also
to ACE inhibitois. May be acute and chionic. In
chionic idiopathic uiticaiia, histamine deiived
fiom mast cells in the skin is consideied the
majoi mediatoi. Eicosanoids and neuiopeptides
may also play a pait in pioducing the lesions.
Nvn-1mmune Cvntuct UrtIcurIu Due to
diiect effects of exogenous uiticants penetiating
into skin oi blood vessels. Localized to site of
contact. Soibic acid, benzoic acid in eye solu-
tions and foods, cinnamic aldehydes in cosmet-
ics, histamine, acetylcholine, seiotonin in nettle
stings.
ürtìcarìa Assocìated wìth vascu|ar[Coooectìve
Iìssue Autoìmmuoe 0ìsease Uiticaiial lesions
may be associated with systemic lupus eiy-
thematosus (SLE) and Sjögien syndiome. How-
evei, in most instances they iepiesent uiticaiial
vasculitis (page 407). This is a foim of cutane-
ous vasculitis associated with uiticaiial skin le-
sions that peisist foi >12 to 24 h. Slow changes
in size and configuiation, can be associated with
puipuia, and can show iesidual pigmentation
due to hemosideiin aftei involution (see Fig.
14-41). Often associated with hypocomplemen-
temia and ienal disease.
0ìstìoct Ao¿ìoedema (± ürtìcarìa) Syodromes
HeredItury AngIvedemu (HAE) A seiious au-
tosomal dominant disoidei; may follow tiauma
(physical and emotional). Angioedema of the
face (Fig. 14-12) and extiemities, episodes of
laiyngeal edema, and acute abdominal pain
caused by angioedema of the bowel wall pie-
senting as suigical emeigency. Uiticaiia iaiely
occuis. Laboiatoiy abnoimalities involve the
complement system: decieased levels of C1-
esteiase inhibitoi (85%) oi dysfunctional in-
hibitoi (15%), low C4 value in the piesence of
noimal C1 and C3 levels. Angioedema iesults
fiom biadykinin foimation, since C1-esteiase
inhibitoi is also the majoi inhibitoi of the
Hageman factoi and kallikiein, the two enzymes
iequiied foi kinin foimation. Episodes can be
life thieatening.
AngIvedemu-UrtIcurIu-EvsInvphI|Iu Syndrvme
Seveie angioedema, only occasionally with piu-
iitic uiticaiia, involving the face, neck, extiemi-
ties, and tiunk that lasts foi 7-10 days. Theie
is fevei and maiked inciease in noimal weight
(incieased by 10-18%) owing to fluid ieten-
tion. No othei oigans aie involved. Laboia-
toiy abnoimalities include stiiking leukocytosis
(20,000-70,000/µL) and eosinophilia (60-80%
eosinophils), which aie ielated to the seveiity
of attack. Theie is no family histoiy. This con-
dition is iaie, piognosis is good.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Edema of the deimis oi
subcutaneous tissue, dilatation of venules but
no evidence of vasculai damage. Mast cell
degianulation. The piedominant peiivasculai
inflammatoiy cell types aie activated lym-
phocytes of the T helpei phenotype.
Sero|o¿y Seaich foi hepatitis B-associated
antigen, assessment of the complement sys-
tem, assessment of specific IgE antibodies by
iadioalleigosoibent test (RAST), anti-Fc RI
autoantibodies. Seiology foi lupus and Sjö-
gien syndiome. Autologous seium skin test foi
autoimmune uiticaiia.
hemato|o¿y The eiythiocyte sedimentation
iate (ESR) is often elevated in uiticaiial vas-
culitis, and theie may be hypocomplemen-
temia; tiansient eosinophilia in uiticaiia fiom
ieactions to foods, paiasites, and diugs; high
levels of eosinophilia in the angioedema-uiti-
caiia-eosinophilia syndiome.
Comp|emeot Studìes Scieening foi functional
C1 inhibitoi in HAE.
ü|trasooo¿raphy Foi eaily diagnosis of bowel
involvement in HAE; if abdominal pain is
piesent, this may indicate edema of the bowel.
Farasìto|o¿y Stool specimen foi piesence of
paiasites.
0IACN0SIS
A detailed histoiy (pievious diseases, diugs,
foods, paiasites, physical exeition, solai expo-
suie) is of utmost impoitance. Histoiy should
diffeientiate between ìy¡e o[ |esíons -uiticaiia,
angioedema, oi uiticaiia - angioedema; Jura-
ìíon o[ |esíons (<1 h oi 1 h), ¡ruríìus , ¡aín
on walking (in foot involvement), [|us|íng ,
|urníng , and w|ee:íng (in cholineigic uiti-
caiia). Fe·er in seium sickness and in the
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 363
FICük£ 14-10 ürtìcarìa: dermo¿raphìsm u|||c+||+ +º || +ppe+|ed 5 r|u +||e| ||e p+||eu| W+º ºc|+|c|ed ou ||e
|+c|. I|e p+||eu| |+d e\pe||euced çeue|+||/ed p|u|||uº |o| ºe.e|+| rou||º W||| uo ºpou|+ueouº|, occu|||uç u|||c+||+.
FICük£ 14-11 Cho|ìoer¿ìc urtìcarìa 'r+|| u|||c+||+| p+pu|eº ou uec| occu|||uç W||||u !0 r|u o| .|ço|ouº
e\e|c|ºe. |+pu|+| u|||c+||+| |eº|ouº +|e |eº| ºeeu uude| º|de ||ç|||uç.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 364
angioedema-uiticaiia-eosinophilia syndiome;
in angioedema, |oarseness , sìríJor , Jys¡nea . Àr-
ì|ra|gía (seium sickness, uiticaiial vasculitis),
a|Jomína| to|ít|y ¡aín in HAE. A caieful his-
toiy of medications including penicillin, aspi-
iin, nonsteioidal anti-inflammatoiy diugs, and
ACE inhibitois should be obtained. Autoim-
mune uiticaiia is tested by the autologous se-
ium skin test and deteimination of anti-Fc RI
antibody .
Deimogiaphism is evoked by stioking the
skin; piessuie uiticaiia is tested by application
of piessuie (weight) peipendiculai to the skin;
vibiation angioedema by a vibiatoiy stimulus,
like iubbing the back with a towel. If ¡|ysíta|
urìítaría is suspected, appiopiiate challenge test-
ing should be peifoimed. C|o|ínergít urìítaría
can best be diagnosed by exeicise to sweating
and intiacutaneous injection of acetylcholine
oi mecholyl, which will pioduce miciopapulai
whealing. So|ar urìítaría is veiified by testing
with UVB, UVA, and visible light. Co|J urìítaría
is veiified by a wheal iesponse to the application
to the skin of an ice cube oi a test tube containing
ice watei. If uiticaiial wheals do not disappeai in
24 h, uiticaiial vasculitis should be suspected
IMAC£ 14-1 App|o+c| |o ||e p+||eu| W||| u|||c+||+/+uç|oeder+. ACE +uç|o|euº|u·cou.e|||uç eu/,re, |çE,
|rruuoç|o|u||u E, ||n, |u|||||o|, , dec|e+ºed. '0ukCE. ||or A| K+p|+u, |u K wo||| e| +| (edº). |·|c¤c|··:|´º
|-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- 1|| ed. |eW \o||, \cC|+W·n|||, 2003, p !!9.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 365
and a biopsy done. The peison with angíoeJema-
urìítaría-eosíno¡|í|ía synJrome has high fevei,
high leukocytosis (mostly eosinophils), a stiiking
inciease in body weight due to ietention of wa-
tei, and a cyclic pattein that may occui and iecui
ovei a peiiod of yeais. HereJíìary angíoeJema
has a positive family histoiy and is chaiacteiized
by angioedema of the face and extiemities as the
iesult of tiauma, abdominal pain, and decieased
levels of C4 and C1-esteiase inhibitoi .
A piactical appioach to the diagnosis of
uiticaiia/angioedema is shown in Image 14-1.
C0ükS£ AN0 Fk0CN0SIS
Half the patients with uiticaiia alone aie fiee of
lesions in 1 yeai, but 20% have lesions foi >20
yeais. Piognosis is good in most syndiomes
except HAE, which may be fatal if untieated.
MANAC£M£NI
Freveotìoo Tiy to pievent attacks by elimina-
tion of etiologic chemicals oi diugs: aspiiin and
food additives, especially in chionic iecuiient
uiticaiia-iaiely successful; pievent tiiggei in
physical uiticaiias.
Aotìhìstamìoes H
1
blockeis, e.g., hydioxyzine,
teifenadine; oi loiatadine, cetiiizine, fexofena-
dine. 180 mg/d of fexofenadine oi 10-20
mg/d of loiatadine usually contiols most cases
of chionic uiticaiia, but cessation of theiapy
usually iesults in a iecuiience; if they fail,
H
1
and H
2
blockeis (cimetidine) and/oi mast
cell-stabilizing agents (ketotifen). Doxepin, a
tiicyclic antidepiessant with maiked H
1
an-
tihistaminic activity, is valuable when seveie
uiticaiia is associated with anxiety and depies-
sion.
Fredoìsooe In atuìe uiticaiia with angio-
edema; also foi angioedema-uiticaiia-
eosinophilia syndiome.
0aoato| or Staooto|o| Long-teim theiapy
foi heieditaiy angioedema; watch out foi hii-
sutism, iiiegulai menses; whole fiesh plasma
oi C1-esteiase inhibitoi in the acute attack. A
veiy effective biadikin-B
2
-ieceptoi antagonist
foi subcutaneous application is now available
in Euiope (Icatibant).
0ther In t|ronít íJío¡aì|ít oi auìoímmune ui-
ticaiia, if no iesponse to antihistamines: switch
to cyclospoiine and tapei giadually, if gluco-
coiticoids aie contiaindicated oi if side effects
occui.
FICük£ 14-12 heredìtary ao¿ìoedema â. 'e.e|e eder+ o| ||e |+ce du||uç +u ep|ºode |e+d|uç |o ç|o·
|eºque d|º||çu|ereu|. 8. Auç|oeder+ W||| ºu|º|de W||||u |ou|º. I|eºe +|e ||e uo|r+| |e+|u|eº o| ||e p+||eu|. I|e
p+||eu| |+d + poº|||.e |+r||, ||º|o|, +ud |+d ru|||p|e º|r||+| ep|ºodeº |uc|ud|uç co||c|, +|dor|u+| p+|u.
â
8
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 366
£FI0£MI0I0C¥
A¿e oI 0oset Thiid and fouith decades.
Freva|eoce Highest in Tuikey (80-420
patients in 100,000), Japan, Southeast Asia, the
Middle East, southein Euiope. Raie in noithein
Euiope, United States (0.12-0.33 in 100,000).
Sex Males > females, but dependent on ethnic
backgiound.
FAIh0C£N£SIS
Etiology unknown. In the eastein Meditei-
ianean and East Asia, HLA-B5 and HLA-B51
association; in the United States and Euiope,
no consistent HLA association. The lesions aie
the iesult of leukocytoclastic (acute) and lym-
phocytic (late) vasculitis.
CIINICAI MANIF£SIAII0N
Painful ulceis eiupt in a cyclic fashion in the
oial cavity and/oi genital mucous membianes.
Oiodynophagia and oial ulceis may peisist/
iecui weeks to months befoie othei symptoms
appeai.
Skìo aod Mucous Membraoes Aphthvus U|cers
Punched-out ulceis (3 to >10 mm) with iolled
oi oveihanging boideis and neciotic base
(Fig. 14-13); ied iim; occui in ciops (2-10) on
oial mucous membiane (100%) (Fig. 14-13;
see also Fig. 34-xx), vulva, penis, and sciotum
(Figs. 14-14, 14-15, 35-15); veiy painful.
Erythemu Nvdvsum-LI|e LesIvns Painful in-
flammatoiy nodules on the aims and legs
(40%) (see Section 7 and Fig. 7-23).
Other Inflammatoiy pustules, supeificial
thiombophlebitis (see Fig. 16-6À), ín[|amma-
ìory ¡|aques iesembling those in Sweet syndiome
k+|e, Wo||dW|de occu||euce, |u| º||ouç|, .+||+||e
e||u|c p|e.+|euce.
|| |º + pe|p|e\|uç ru|||º,º|er .+ºcu||||c d|ºe+ºe
W||| ru|||o|ç+u |u.o|.ereu|.
\+|u º,rp|orº +|e |ecu||eu| o|+| +p|||ouº
u|ce|º, çeu||+| u|ce|º, e|,||er+ uodoºur, ºupe|·
||c|+| |||or|op||e||||º, º||u puº|u|eº, |||doc,c||||º,
+ud poº|e||o| u.e|||º.
Add|||ou+| º,rp|orº r+, |e +|||||||º, ep|d|·
d|r|||º, ||eocec+| u|ce|+||ouº, .+ºcu|+| +ud ceu||+|
ue|.ouº º,º|er (C|') |eº|ouº.
C||ou|c |e|+pº|uç p|oç|eºº|.e cou|ºe W||| po|eu·
||+||, poo| p|oçuoº|º.
8£hÇ£I 0IS£AS£ |C|·9 . ¹19.+
°
|C|·¹0 . \!5.2
(acute febiile neutiophilic deimatosis) (see Fig.
7-30), ¡yoJerma gangrenosum-|í|e |esíons (see
Fig. 7-26), ¡a|¡a||e ¡ur¡urít |esíons of neciotiz-
ing vasculitis (see Fig. 14-34).
Systemìc Fìodìo¿s Eyes Leading cause of
moibidity. Posteiioi uveitis, anteiioi uveitis,
ietinal vasculitis, vitieitis, hypopyon, secondaiy
cataiacts, glaucoma, neovasculai lesions.
Muscu|vs|e|etu| Noneiosive, asymmetiic oli-
goaithiitis.
Neurv|vgIc Onset delayed, occuiiing in
one quaitei of patients. Meningoencephalitis,
benign intiacianial hypeitension, cianial neive
palsies, biainstem lesions, pyiamidal/extiapy-
iamidal lesions, psychosis.
Vuscu|ur Aneuiysms, aiteiial occlusions,
venous thiombosis, vaiices; hemoptysis. Coio-
naiy vasculitis: myocaiditis, coionaiy aiteiitis,
endocaiditis, valvulai disease.
G1 Truct Aphthous ulceis thioughout.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Leukocytoclastic vasculitis
with fibiinoid neciosis of blood vessel walls in
acute eaily lesions; lymphocytic vasculitis in
late lesions.
Father¿y Iest Positive patheigy test iead by
physician at 24 oi 48 h, aftei skin punctuie with
a steiile needle. Leads to inflammatoiy pustule.
hIA Iypìo¿ Significant association with HLA-
B5 and HLA-B51, in Japanese, Koieans, and
Tuiks, and in the Middle East.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Diagnosis is made accoiding to the Revised
Inteinational Ciiteiia foi Behçet disease
(Image 14-2).
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 36T
FICük£ 14-13 8ehçet dìsease 0|+| +p|||ouº u|ce|º. â. I|eºe +|e ||ç||, p+|u|u|, puuc|ed·ou| u|ce|º W|||
+ uec|o||c |+ºe ou ||e |ucc+| rucoº+ +ud |oWe| +ud uppe| |o|u|\ |u |||º 23·,e+|·o|d Iu|||º| r+|e. 8. A puuc|ed·
ou| u|ce| ou ||e |ouçue o| +uo||e| p+||eu|.
â
8
FICük£ 14-14 8ehçet dìsease: ¿eoìta| u|cers \u|||p|e |+|çe +p|||ouº·|,pe u|ce|º ou ||e |+||+| +ud pe||·
ue+| º||u. |u +dd|||ou, |||º 25·,e+|·o|d p+||eu| o| Iu|||º| e\||+c||ou |+d +p|||ouº u|ce|º |u ||e rou|| +ud p|e.|·
ouº|, e\pe||euced +u ep|ºode o| u.e|||º.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 368
0ìIIereotìa| 0ìa¿oosìs Ora| anJ geníìa| u|ters :
Viial infection ¦heipes simplex viius (HSV),
vaiicella-zostei viius (VZV)], hand-foot-and-
mouth disease, heipangina, chancie, histoplas-
mosis, squamous cell caicinoma.
C0ükS£ AN0 Fk0CN0SIS
Highly vaiiable couise, with iecuiiences and
iemissions; the mouth lesions aie always
piesent; iemissions may last foi weeks, months,
oi yeais. In the eastein Mediteiianean and East
Asia, seveie couise, one of the leading causes
of blindness. With CNS involvement, theie is a
highei moitality iate.
MANAC£M£NI
Aphthous ü|cers Potent topical glucocoiti-
coids. Intialesional tiiamcinolone, 3-10 mg/mL,
injected into ulcei base. Thalidomide, 50-100
mg PO in the evening. Colchicine, 0.6 mg PO 2
to 3 times a day. Dapsone, 50-100 mg/d PO.
Systemìc Iovo|vemeot Piednisone with oi
without azathiopiine, cyclophosphamide, aza-
thiopiine alone, chloiambucil, cyclospoiine.
IMAC£ 14-2 ke.|ºed |u|e|u+||ou+| C|||e||+ |o| Be|(e| ||ºe+ºe (|u|e|u+||ou+| Ie+r |o| ||e ke.|º|ou o| |CB|,
coo|d|u+|o|. |. |+.+|c||) +cco|d|uç |o (A) ||e c|+ºº|||c+||ou ||ee |o|r+|, +ud (8) ||e ||+d|||ou+| |o|r+| AB|,
Ad+r+u||+deº·Be|(e| d|ºe+ºe, Cu, çeu||+| u|ce|, 0A, o|+| +p|||ouº u|ce|. '0ukCE. CC /ou|ou||º. Ad+r+u||+deº
Be|(e| d|ºe+ºe, |u K wo||| e| +| (edº). |·|c¤c|··:|´º |-·¤c|¤|¤¸, ·¤ C-¤-·c| !-!·:·¤- 1|| ed. |eW \o||, \cC|+W·
n|||, 2003, pp ¹o20-¹o22.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 369
FICük£ 14-15 8ehçet dìsease A |+|çe, puuc|ed·ou| u|ce| ou ||e ºc|o|ur o| + +0·,e+|·o|d Ko|e+u. I|e
p+||eu| +|ºo |+d +p|||ouº u|ce|º |u ||e rou|| +ud puº|u|eº ou ||e |||ç|º +ud |u||oc|º.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 3T0
£FI0£MI0I0C¥ AN0 £II0I0C¥
Raie. Incidence >6 cases pei million, but this
is based on hospitalized patients and does not
include individuals without muscle involve-
ment. Juvenile and adult (>40 yeais) onset.
£tìo|o¿y Unknown. In peisons >55 yeais of
age, may be associated with malignancy.
C|ìoìca| Spectrum Ranges fiom DM with only
cutaneous inflammation (amyopathic DM) to
polymyositis with only muscle inflammation.
Cutaneous involvement occuis in 30-40% of
adults and 95% of childien with deimato-
myositis/polymyositis. Foi classification, see
Table 14-2.
CIINICAI MANIF£SIAII0N
± Photosensitivity. Manifestations in skin
disease may piecede myositis oi vice veisa;
often, both aie detected at the same time. Mus-
cle weakness, difficulty in iising fiom supine
position, climbing staiis, iaising aims ovei
head, tuining in bed. Dysphagia; buining and
piuiitus of the scalp.
Skìo Iesìoos Peiioibital heliotiope (ieddish
puiple) flush, usually associated with some
degiee of edema (Fig. 14-16). May extend to
involve scalp (- nonscaiiing alopecia), entiie
face (Fig. 14-17À), uppei chest, and aims.
In addition, papulai deimatitis with vaiying
degiees of violaceous eiythema in the same
sites. Flat-topped, violaceous papules (Gottion
papule/sign) with vaiious degiees of atiophy
on the nape of the neck and shouldeis and
ovei the knuckles and inteiphalangeal joints
(Fig. 14-17B). Noìe : In lupus, lesions usually
occui in the inteiaiticulai iegion of the fingeis
(see Fig. 14-21À). Peiiungual eiythema with
|e|r+|or,oº|||º (|\) |º + º,º|er|c d|ºe+ºe |e·
|ouç|uç |o ||e |d|op+|||c |u||+rr+|o|, r,op+|||eº,
+ |e|e|oçeueouº ç|oup o| çeue||c+||, de|e|r|ued
+u|o|rruue d|ºe+ºeº |+|çe||uç ||e º||u +ud/o|
º|e|e|+| ruºc|eº.
|\ |º c|+|+c|e||/ed |, .|o|+ceouº (|e||o||ope)
|u||+rr+|o|, c|+uçeº +/÷ eder+ o| ||e e,e||dº
+ud pe||o||||+| +|e+, e|,||er+ o| ||e |+ce, uec|,
+ud uppe| ||uu|, +ud ||+|·|opped .|o|+ceouº p+·
pu|eº o.e| ||e |uuc||eº.
|| |º +ººoc|+|ed W||| po|,r,oº|||º, |u|e|º||||+| pueu·
rou|||º, +ud r,oc+|d|+| |u.o|.ereu|.
|\ W|||ou| r,op+||, (+r,op+|||c |\) +ud
po|,r,oº|||º W|||ou| º||u |u.o|.ereu|.
lu.eu||e |\ |uuº + d|||e|eu| cou|ºe +ud |º +ººoc|·
+|ed W||| .+ºcu||||º +ud c+|c|uoº|º.
Adu||·ouºe| |\ r+, |e +ººoc|+|ed W||| |u|e|u+|
r+||çu+uc,.
||oçuoº|º |º çu+|ded.
0£kMAI0M¥0SIIIS |C|·9 . 1¹0.!
°
|C|·¹0 . \!!.0
tel-angiectasia, thiombosis of capillaiy loops, in-
faictions. Lesions ovei elbows and knuckles
may evolve into eiosions and ulceis that heal
with stellate scaiiing (paiticulaily in juvenile
DM with vasculitis). Long-lasting lesions may
evolve into poikilodeima (mottled discoloia-
tion with ied, white, and biown) (Fig. 14-18).
Calcification in subcutaneous/fascial tissues
common latei in couise of juvenile DM, pai-
ticulaily about elbows, tiochanteiic, and iliac
iegion (calcinosis cutis); may evolve into calci-
nosis univeisalis.
Musc|e ± Muscle tendeiness, ±muscle atiophy.
Piogiessive muscle weakness affecting pioxi-
mal/limb giidle muscles. Difficulty oi inability
to iise fiom sitting oi supine position without
using aims. Difficulty in iaising aims above
head and difficulty in climbing staiis. Inteicos-
tal muscles: difficulty in bieathing.
Occasional involvement of facial/bulbai, pha-
iyngeal, and esophageal muscles. Deep tendon
ieflexes within noimal limits.
0ther 0r¿aos Inteistitial pneumonitis, cai-
diomyopathy, aithiitis, paiticulaily in juvenile
DM (20-65%).
0ìsease Assocìatìoo Patients >50 yeais of age
with DM have a highei than expected iisk
foi malignancy, paiticulaily ovaiian cancei in
females. Also caicinoma of the ovaiy, bieast,
bionchopulmonaiy, and GI tiact. Most canceis
detected within 2 yeais of diagnosis.
IA80kAI0k¥ £XAMINAII0NS
Chemìstry Duiing acute active phase: eleva-
tion of cieatine phosphokinase (65%), which
is most specific foi muscle disease; also, of
aldolase (40%), lactate dehydiogenase, glutamic
oxaloacetic tiansaminase.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 3T1
Autoaotìbodìes Autoantibodies to 155 kDa
and/oi Se in 80% to 140 kDa in 58% and to Jo-
1 in 20% (both have a high specificity foi DM)
and to (low specificity) antinucleai antibodies
(ANA, nucleai and speckled pattein) in 40%.
ürìoe Elevated 24-h cieatine excietion (>200
mg/24 h). Note: can also be elevated in gluco-
coiticoid myopathy.
£| ectromyo¿raphy Incieased iiiitability
on inseition of electiodes, spontaneous
fibiillations, pseudomyotonic dischaiges, pos-
itive shaip waves: excludes neuiomyopathy.
With evidence of deneivation, suspect coexist-
ing tumoi.
MkI MRI of muscles ieveals focal lesions.
£CC Evidence of myocaiditis; atiial, ventiicu-
lai iiiitability; atiioventiiculai block.
X-kay C|esì: ± inteistitial fibiosis. Eso¡|agus :
ieduced peiistalsis.
Fatho|o¿y S|In Flattening of epideimis,
hydiopic degeneiation of basal cell layei, edema
of uppei deimis, scatteied inflammatoiy infil-
tiate, PAS-positive fibiinoid deposits at deimal-
epideimal junction and aiound uppei deimal
capillaiies, accumulation of acid mucopolysac-
chaiides in deimis (all these aie compatible
with DM but aie not diagnostic).
Musc|e Biopsy shouldei/pelvic giidle; one that
is weak oi tendei, i.e., deltoid, supiaspinatus,
gluteus, quadiiceps aftei maiking by EMG oi
IA8I£ 14-2 Comprehensive C|assification
of |diopathic |nf|ammatory
0ermatomyopathies
0ermatomyosìtìs (0M)
Adu|| ouºe|
C|+ºº|c |\. +|oue, W||| r+||çu+uc,, +º p+|| o| +u
o.e||+p couuec||.e ||ººue d|ºo|de|
C||u|c+||, +r,op+|||c |\. +r,op+|||c |\. |,po·
r,op+|||c |\
lu.eu||e ouºe|
C|+ºº|c |\
C||u|c+||, +r,op+|||c |\. +r,op+|||c |\, |,po·
r,op+|||c |\
Fo|ymyosìtìs (FM)
|\ +|oue
|\ +º p+|| o| +u o.e||+p couuec||.e ||ººue d|ºo|de|
|\ +ººoc|+|ed W||| |u|e|u+| r+||çu+uc,
c
Ioc|usìoo 8ody Myosìtìs
0ther C|ìoìca|-Fatho|o¿ìc Sub¿roups oI Myosìtìs
|oc+| r,oº|||º Eoº|uop||||c r,oº|||º
||o|||e|+||.e r,oº|||º C|+uu|or+|ouº r,oº|||º
0||||+| r,oº|||º
c
A|||ouç| popu|+||ou·|+ºed Eu|ope+u º|ud|eº |+.e uoW c|e+||,
cou|||red ||+| +du||·ouºe| c|+ºº|c |\ |º +ººoc|+|ed W||| + º|çu|||c+u|
||º| |o| |u|e|u+| r+||çu+uc,, || ºuc| + |e|+||ouº||p e\|º|º |o| |\, || |º
ruc| We+|e|.
FICük£ 14-16 0ermatomyosìtìs ne||o||ope (|edd|º| pu|p|e) e|,||er+ o| uppe| e,e||dº +ud eder+ o| ||e
|oWe| ||dº. I||º 55·,e+|·o|d |er+|e |+d e\pe||euced ºe.e|e ruºc|e We+|ueºº o| ||e º|ou|de| ç||d|e +ud p|eºeu|ed
W||| + |urp |u ||e ||e+º| ||+| p|o.ed |o |e c+|c|uor+.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 3T2
FICük£ 14-1T 0ermatomyosìtìs â. \|o|+ceouº e|,||er+ +ud eder+ ou ||e |+ce, p+|||cu|+||, |u ||e pe||o||||+|
+ud r+|+| |eç|ouº. I|e p+||eu| cou|d |+|e|, |||| ||º +|rº +ud cou|d uo| c||r| º|+||º. ne |+d pu|rou+|, c+|c|uor+. 8.
\|o|+ceouº e|,||er+ +ud Co|||ou p+pu|eº ou ||e do|º+ o| ||e |+udº +ud ||uçe|º, eºpec|+||, o.e| ||e re|+c+|pop|+·
|+uçe+| +ud |u|e|p|+|+uçe+| jo|u|º, ||e ||ç||·p|o|ec|ed +|e+º o| ||e |o|e+|rº o| |||º 23·,e+|·o|d r+|e W||| ºe.e|e ruºc|e
We+|ueºº We|e uo| |u.o|.ed. |e||uuçu+| e|,||er+ +ud |e|+uç|ec|+º|º. I||º p+||eu| |+|e| de.e|oped ºe.e|e c+|c|uoº|º cu||º.
â
8
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 3T3
MRI. Histology-segmental neciosis within
muscle fibeis with loss of cioss-stiiations; waxy/
coagulative type of eosinophilic staining; with oi
without iegeneiating fibeis; inflammatoiy cells,
histiocytes, maciophages, lymphocytes, plasma
cells. Vasculitis is seen in juvenile DM. MRI-
guided needle biopsy of muscle may ieplace con-
ventional muscle biopsy in the futuie.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Skin signs plus pioximal muscle weakness with
two of thiee laboiatoiy ciiteiia, i.e., elevated
seium °muscle enzyme" levels, chaiacteiistic
electiomyogiaphic changes, diagnostic muscle
biopsy. Diffeiential diagnosis is to seboiiheic
deimatitis, lupus eiythematosus, mixed con-
nective tissue disease, steioid myopathy, tiichi-
nosis, toxoplasmosis.
C0ükS£ AN0 Fk0CN0SIS
Piognosis guaided but with tieatment, it is
ielatively good except in patients with malig-
nancy and those with pulmonaiy involvement.
With aggiessive immunosuppiessive tieatment
the 8-yeai suivival iate is 70-80%. A bet-
tei piognosis is seen in individuals who ie-
ceive eaily systemic tieatment. The eaily and
aggiessive use of glucocoiticoids has ieduced
the moitality iates in childien to <10%. The
most common causes of death aie malignancy,
infection, caidiac and pulmonaiy disease. Suc-
cessful tieatment of an associated neoplasm
is often followed by impiovement/iesolution
of DM.
MANAC£M£NI
Fredoìsooe 0.5-1 mg/kg body weight pei day,
incieasing to 1.5 mg/kg if lowei dose ineffective.
Tapei when °muscle enzyme" levels appioach
noimal. Best if combined with azathiopiine,
2-3 mg/kg pei day. Noìe: Steioid myopathy may
occui aftei 4-6 weeks of theiapy.
A|teroatìves Methotiexate, cyclophospha-
mide, cyclospoiine, anti-tumoi neciosis factoi
(TNF) agents. High-dose IV immunoglobu-
lin bolus theiapy (2 g/kg body weight given ovei
2 days) at monthly inteivals spaies glucocoiti-
coid doses to achieve oi maintain iemissions.
FICük£ 14-18 0ermatomyosìtìs, juveoì|e ooset, poìkì|oderma I|e|e |º ro|||ed, |e||cu|+| ||oWu|º|
p|çreu|+||ou +ud |e|+uç|ec|+º|+ p|uº ºr+|| W|||e ºc+|º. |o|e º|||+e ou ||oc|+u|e||c +|e+º due |o º,º|er|c ç|ucoco|·
||co|d ||e|+p,.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 3T4
£II0I0C¥
IIk A physiologic phenomenon.
SIk That of associated disoidei; following
amantadine tieatment foi Paikinson disease.
FAIh0C£N£SIS
ILR pattein due to vasospasm oi obstiuction
of peipendiculai aiteiioles, peifoiating deimis
fiom below. Cyanotic peiipheiy of each web of
net caused by deoxygenated blood in suiiound-
ing hoiizontally aiianged venous plexuses.
When factois such as cold cause incieased vis-
cosity oi flow iates in supeificial venous plexus,
fuithei deoxygenation occuis and cyanotic ie-
ticulai pattein becomes moie pionounced. El-
evation of limb decieases intensity of coloi due
to incieased venous diainage. SLR iesults fiom
aiteiiolai disease causing obstiuction to inflow
and blood hypeiviscosity oi fiom obstiuction
to outflow of blood in venules.
CIINICAI MANIF£SIAII0N
Appeaiance oi woisening with cold exposuie.
± Numbness, tingling associated. Woise duiing
wintei months.
Skìo Iesìoos 1LR A puiple/livid discoloia-
tion of skin in netlike pattein (mesh diametei
3 cm) involving laige aieas of lowei oi uppei
extiemities and tiunk and disappeaiing aftei
waiming.
SLR Blotchy, aiboiizing, lightning-like, stai-
buist, oi mottled pattein of cyanosis (Fig.
14-19). Netlike webs aie open (semiciiculai),
and within webs, skin is noimal to pallid and
||.edo |e||cu|+||º (|k) |º + ro|||ed ||u|º| (||.|d)
d|ºco|o|+||ou o| ||e º||u ||+| occu|º |u + ue||||e
p+||e|u. || |º uo| + d|+çuoº|º |u ||ºe|| |u| + |e+c||ou
p+||e|u.
C|+ºº|||c+||ou d|º||uçu|º|eº |e|Weeu
|!·¤¤c||·: |·.-!¤ ·-|·:±|c··º (||k) . + pu|p|e/||.|d
d|ºco|o|+||ou o| ||e º||u |u + ue||||e p+||e|u
d|º+ppe+||uç +||e| W+|r|uç. A p|,º|o|oç|c p|e·
uoreuou. ( ',¤¤¤,¤. cu||º r+|ro|+|+.)
'-:¤¤!c·, (º,¤¤|¤¤c|·:) |·.-!¤ ·-|·:±|c··º ('|k) .
+ pu|p|e d|ºco|o|+||ou occu|||uç |u + º|+||u|º| o|
||ç||u|uç·|||e p+||e|u, ue||||e |u| W||| opeu (uo|
+uuu|+|) reº|eº, roº||,, |u| uo| +|W+,º, cou||ued
|o ||e |oWe| e\||er|||eº +ud |u||oc|º. A |e+c||ou
p+||e|u o||eu |ud|c+||.e o| ºe||ouº º,º|er|c d|º·
e+ºe (I+||e ¹+·!). ( ',¤¤¤,¤. ||.edo |+ceroº+.)
IIv£00 k£IICüIAkIS |C|·9 . ++o.20
°
|C|·¹0 . | 95.0
feels cool. Symmetiic, aims/legs, buttocks; less
commonly, body. On exposuie to cold, livedo
becomes moie pionounced but nevei fades
completely on waiming. It nevei ulceiates.
Noìe : When associated with |í·eJoíJ ·astu|íìís
(see p. 475), ulceiation about ankles and foie-
feet may occui.
Ceoera| £xamìoatìoo Symptoms of undeily-
ing disease in SLR (Table 14-3).
IA80kAI0k¥ £XAMINAII0NS
Iaboratory Vaiies with associated disoideis.
0ermatopatho|o¿y Vasculai pathology of un-
deilying disease.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical diagnosis confiimed by laboiatoiy data
suppoiting diagnosis of associated disoidei.
0ìIIereotìa| 0ìa¿oosìs Cutis maimoiata, ILR
veisus SLR, livedoid vasculitis (see page 475),
eiythema ab igne.
C0ükS£ AN0 Fk0CN0SIS
Couise/piognosis of SLR depends on that of
associated disoidei.
MANAC£M£NI
· Keep fiom chilling. Pentoxifylline (400 mg
PO thiee times a day), low-dose aspiiin, and
hepaiin may be helpful.
· Tieat associated disoidei.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 3T5
IA8I£ 14-3 0isorders Associated with Symptomatic Livedo Reticu|aris
Vasc0|ar 0bstr0ct|oo V|scos|ty 0haoges 0r0gs
A||e|oer|o|| I||or|oc,||er|+ Ar+u|+d|ue
A||e||oºc|e|oº|º |o|,ç|o|u||uer|+ 0u|u|ue
|o|,+||e||||º uodoº+ C|,oç|o|u||uer|+ 0u|u|d|ue
Cu|+ueouº po|,+||e||||º uodoº+ Co|d +çç|u||uer|+
k|eur+|o|d .+ºcu||||º ||ººer|u+|ed |u||+.+ºcu|+|
||.edo|d .+ºcu||||º co+çu|+||ou
'ueddou º,ud|ore |upuº e|,||er+|oºuº
Au||c+|d|o||p|u º,ud|ore
|eu|er|+/|,rp|or+
FICük£ 14-19 Symptomatìc |ìvedo retìcu|arìs A ue||||e, +||o||/|uç p+||e|u ou ||e poº|e||o| |||ç|º +ud
|u||oc|º de||ued |, .|o|+ceouº, e|,||er+|ouº º||e+|º |eºer|||uç ||ç||u|uç. I|e º||u W||||u ||e e|,||er+|ouº
+|e+º |º uo|r+||, p+|e. I||º occu||ed |u + p+||eu| W||| |+|||e |,pe||euº|ou +ud ru|||p|e ce|e||o.+ºcu|+| +||+c|º +ud
W+º ||uº p+||oçuorou|c |o| 'ueddou º,ud|ore.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 3T6
£FI0£MI0I0C¥
Raie but undeidiagnosed.
CIINICAI MANIF£SIAII0N
Skin lesions piecede neuiologic symptoms,
often by yeais.
Skìo Iesìoos These iepiesent classic SLR on
lowei extiemities, buttocks, sometimes aims (Fig.
14-19). ± Angiomatosis (mottled-puiple discol-
oiation of the face and othei paits of the body).
Noìe : Sneddon syndiome is not identical
with antiphospholipid syndiome, although dei-
matologic manifestations (SLR) may be indis-
tinguishable. May be associated with livedoid
vasculitis-in this case, ulceiation may occui
aiound ankles oi acially (see page 475).
A po|eu||+||, |||e·|||e+|eu|uç d|ºe+ºe o| uu|uoWu
e||o|oç, occu|||uç ro|e o||eu |u |er+|eº ||+u
r+|eº +ud r+u||eº||uç r+|u|, |u º||u +º '|k +ud
|u ||e C|'.
Aººoc|+|ed W||| ||+uº|eu| |ºc|er|c +||+c|º +ud
ce|e||o.+ºcu|+| |uºu||.
SN£000N S¥N0k0M£
Neuro|o¿ìc Symptoms Include headaches,
labile hypeitension, tiansient ischemic attacks,
tiansient amnesia, tiansient aphasia, palsy, and
ceiebiovasculai insult.
IA80kAI0k¥ £XAMINAII0N
0ermatopatho|o¿y Endothelitis piolif-
eiation of subendothelial myofibioblasts
vasculai occlusion and fibiosis. Cytotoxic anti-
endothelial cell antibodies in a small peicentage
of patients. Theie may be antiphospholipid
antibodies.
MANAC£M£NI
Longtime low-dose hepaiin, aspiiin.
|E |º ||e deº|çu+||ou o| + ºpec||ur o| d|ºe+ºe
p+||e|uº ||+| +|e ||u|ed |, d|º||uc| c||u|c+| ||ud·
|uçº +ud d|º||uc| p+||e|uº o| ce||u|+| +ud |uro|+|
+u|o|rruu||,.
|E occu|º ro|e corrou|, |u Woreu (r+|e |o
|er+|e |+||o ¹. 9).
|E |+uçeº ||or |||e·|||e+|eu|uç r+u||eº|+||ouº o|
+cu|e º,º|er|c |E ('|E) |o ||e ||r||ed +ud e\c|u·
º|.e º||u |u.o|.ereu| |u c||ou|c cu|+ueouº |E
(CC|E) (|r+çe ¹+·!). \o|e ||+u 35° o| p+||eu|º
W||| |E |+.e º||u |eº|ouº, W||c| c+u |e c|+ºº|||ed
|u|o |E·ºpec|||c +ud ·uouºpec|||c.
Au +|||e.|+|ed .e|º|ou o| C||||+r c|+ºº|||c+||ou o|
|E·ºpec|||c º||u |eº|ouº |º ç|.eu |u I+||e ¹+·+.
Acu|e cu|+ueouº |E (AC|E) |º p|+c||c+||, +|·
W+,º +ººoc|+|ed W||| '|E, ºu|+cu|e cu|+ueouº |E
('C|E) |u +|ou| 50°, +ud c||ou|c cu|+ueouº |E
(CC|E) roº| o||eu |+º ou|, º||u d|ºe+ºe. noW·
e.e|, CC|E |eº|ouº c+u occu| |u '|E.
AC|E +ud 'C|E +|e ||ç||, p|o|oºeuº|||.e.
IüFüS £k¥Ih£MAI0SüS (I£) |C|·9 . o95.+
°
|C|·¹0 . |9!
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 3TT
IA8I£ 14-4 Abbreviated Ci||iam C|assification of Skin Lesions of LE
|. |E·ºpec|||c º||u d|ºe+ºe ¦cu|+ueouº |E
*
(C|E)|
A. Acu|e cu|+ueouº |E ¦AC|E|
¹. |oc+||/ed AC|E (r+|+| |+º|, |u||e|||, |+º|)
2. Ceue|+||/ed AC|E (r+cu|op+pu|+| |upuº |+º|, r+|+| |+º|, p|o|oºeuº|||.e |upuº de|r+||||º)
B. 'u|+cu|e cu|+ueouº |E ¦'C|E|
¹. Auuu|+| 'C|E
2. |+pu|oºqu+rouº 'C|E (d|ººer|u+|ed ||E, ºu|+cu|e d|ººer|u+|ed |E, r+cu|op+pu|+| p|o|oºeuº|||.e |E)
C. C||ou|c cu|+ueouº |E ¦CC|E|
¹. C|+ºº|c d|ºco|d |E ¦||E|. (+) |oc+||/ed ||E, (|) çeue|+||/ed ||E
2. n,pe|||op||c/.e||ucouº ||E
!. |upuº p|o|uuduº
+. \ucoº+| ||E. (+) o|+| ||E, (|) coujuuc||.+| ||E
5. |upuº |ur|duº (u|||c+||+| p|+que o| |E)
o. C|||||+|uº |E (c|||||+|uº |upuº)
1. ||c|euo|d ||E (|E/||c|eu p|+uuº o.e||+p)
||. |E·uouºpec|||c º||u d|ºe+ºe
I|eºe |+uçe ||or uec|o||/|uç +ud u|||c+||+| .+ºcu||||º |o ||.edo |e||cu|+||º, k+,u+ud p|euoreuou, de|r+| ruc|uoº|º,
+ud |u||ouº |eº|ouº |u |E.
A||e|u+||.e o| º,uou,rouº |e|rº +|e ||º|ed |u p+|eu||eºeº, +|||e.|+||ouº +|e |ud|c+|ed |u ||+c|e|º.
'0ukCE. kep||u|ed +ud rod|||ed ||or k| 'ou||e|re| W||| pe|r|ºº|ou ||or '|oc||ou lou|u+|º, \+cr|||+u ||eºº, ||d.
IMAC£ 14-3 I|e ºpec||ur o| |upuº e|,||er+|oºuº, +º eu.|º+çed |, ||e |+|e ||. l+reº |. C||||+r. I|e |e||
corp||ºeº coud|||ouº ||+| de||ue cu|+ueouº d|ºe+ºe ou|, +ud || c+u |e ºeeu ||+| c||ou|c cu|+ueouº |upuº e\|eudº
|u|o ||e º,º|er|c d|ºe+ºe ºec||ou. I||º |º +|ºo ||ue |o| |upuº p|o|uuduº (|upuº p+uu|cu||||º) +ud ºu|+cu|e cu|+ue·
ouº |upuº, W|e|e+º +cu|e cu|+ueouº |upuº |º c|+|+c|e||º||c |o| º,º|er|c d|ºe+ºe ou|,. I|e |o||or º|oWº ||+|
|rruue corp|e\ d|ºe+ºe dor|u+|eº º,º|er|c d|ºe+ºe +ud ce||·red|+|ed |rruu||, (C\|) |º p|edor|u+u| |u ||e
cu|+ueouº d|ºe+ºe r+u||eº|+||ouº.
C.M.Ì
Ìmmune
complex
disease
Chronic
cutaneous
LE
Acute
cutaneous
LE
s
u
o
e
n
a
u
c
e
t
u
c
a
b
u
S
LE
u rof n P du E s L
Y
L
N
O
E
S
A
E
S
Ì
D
S
U
O
E
N
A
T
U
C
S
Y
S
T
E
M
Ì
C
D
Ì
S
E
A
S
E
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 3T8
£FI0£MI0I0C¥
Freva|eoce
Noithein Euiopeans to moie than 200/100,000
A¿e oI 0oset
Sex
kace Moie common in blacks.
Frecìpìtatìo¿ Factors
sunlight (UVR) is the most effective piecipitat-
ing factoi. An SLE syndiome can be induced by
diugs (hydialazine, ceitain anticonvulsants, and
piocainamide), but iash is a ielatively uncom-
mon featuie of diug-induced SLE.
CIINICAI MANIF£SIAII0N
(chionic). Sunlight may cause an exaceibation
of SLE (36%). Piuiitus, buining of skin lesions.
Fatigue (100%), fevei (100%), weight loss, and
malaise. Aithialgia oi aithiitis, abdominal pain,
Skìo Iesìoos
14-4) in the acute phases of the disease and SCLE
and CCLE lesions. Wheieas ACLE lesions occui
only in acute oi subacute SLE, SCLE and CCLE
lesions aie piesent in subacute and chionic SLE
but may also occui in acute SLE. ACLE lesions
ACI£ Butter]|y Rush
ent, maculai butteifly eiuption on the face
(Fig. 14-20), shaiply defined with fine scaling;
Generu|Ized
oi uiticaiial lesions on the face, on the doisa of
hands (Fig. 14-21À
Others Bu||ae
flaies). Pa¡u|es sta|y ¡|aques
(Fig. 14-22) and JístoíJ ¡|aques
(Fig. 14-23), piedominantly on the face and
I||º ºe||ouº ru|||º,º|er +u|o|rruue d|ºe+ºe
|º |+ºed ou po|,c|ou+| B ce|| |rruu||,, W||c|
|u.o|.eº couuec||.e ||ººue +ud ||ood .eººe|º.
\o|e corrou |u pe|ºouº W||| ||+c| A|||c+u |e|||·
+çe, r+|e |o |er+|e |+||o ¹. 9.
I|e c||u|c+| r+u||eº|+||ouº |uc|ude |e.e| (90°),
º||u |eº|ouº (35°), +|||||||º, C|', |eu+|, c+|d|+c,
+ud pu|rou+|, d|ºe+ºe.
'||u |eº|ouº +|e ||oºe o| AC|E +ud 'C|E, uo|
uucorrou|, o| CC|E.
'|E r+, uucorrou|, de.e|op |u p+||eu|º W|||
CC|E, ou ||e o||e| |+ud, |eº|ouº o| CC|E +|e
corrou |u '|E (|r+çe ¹+·!).
S¥SI£MIC IüFüS £k¥Ih£MAI0SüS |C|·9 . 1¹0.0
°
|C|·¹0 . |9!
on the aims and scalp. Eiythematous, some-
times violaceous, slightly scaling, densely set
and ton[|uenì ¡a¡u|es
fingei, usually with spaiing of the aiticulai
iegions (Fig. 14-21À). Note diffeience to
deimatomyositis (Fig. 14-17B). Pa|mar ery-
ì|ema B),
naí|[o|J ìe|angíetìasías
thema, edema of the peiiungual skin, (see
Fig. 33-26). °Palpable" puipuia (vasculitis),
lowei extiemities (see Fig. 14-34). Urìítaría|
|esíons
haìr
Mucous Membraoes
puiic neciotic lesions on palate (80%), buccal
SItes v] PredI|ectIvn
piefeientially in light-exposed sites. Face (80%);
scalp (Fig. 32-17) (discoid lesions); piesteinal,
shouldeis; doisa of the foieaims, hands, fingeis,
fingeitips (Fig. 33-31) (Image 14-4).
£xtracutaoeous Mu|tìsystem Iovo|vemeot
(50%), peiicaiditis (20%), pneumonitis (20%),
gastiointestinal (due to aiteiitis and steiile
peiitonitis), hepatomegaly (30%), myopathy
(30%), splenomegaly (20%), lymphadenopa-
thy (50%), peiipheial neuiopathy (14%), CNS
disease (10%), seizuies oi oiganic biain disease
(14%).
IA80kAI0k¥ £XAMINAII0NS
Fatho|o¿y S|In
faction degeneiation of the deimal-epideimal
junction, edema of the deimis, deimal lym-
phocytic infiltiate, and fibiinoid degeneiation
of the connective tissue and walls of the blood
vessels.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 3T9
FICük£ 14-20 Acute systemìc |upus erythematosus B||ç|| |ed, º|+|p|, de||ued e|,||er+ W||| º||ç||
eder+ +ud r|u|r+| ºc+||uç |u + '|u||e|||, p+||e|u¨ ou ||e |+ce. I||º |º ||e |,p|c+| 'r+|+| |+º|.¨ |o|e +|ºo ||+| ||e
p+||eu| |º |er+|e +ud ,ouuç.
IMAC£ 14-4 ||ed||ec||ou º||eº o| cu|+ueouº |upuº e|,||er+|oºuº.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 380
1mmunv]|uvrescence v] S|In The lupus
band test (LBT, diiect immunofluoiescence
demonstiating IgG, IgM, C3) shows gianulai
oi globulai deposits of immune ieactants in a
bandlike pattein along the deimal-epideimal
junction. Positive in lesional skin in 90% and
in the clinically noimal skin (sun-exposed,
70-80%; non-sun-exposed, 50%).
Sero|o¿y ANA positive (>95%); peiipheial
pattein of nucleai fluoiescence. Anti-double-
stiand DNA antibodies, anti-Sm antibodies,
and iRNP antibodies specific foi SLE; low levels
of complement (especially with ienal involve-
ment). Anticaidiolipin autoantibodies (lupus
anticoagulant) in a specific subset (anticaidioli-
pin syndiome); SS-A(Ro) autoantibodies have
a low specificity foi SLE but aie specific in the
subset of SCLE (see below) (Table 14-5).
hemato|o¿y Anemia ¦noimocytic, noimo-
chiomic, oi iaiely, hemolytic Coombs-positive,
leukopenia (>4000/µL)], lymphopenia, thiom-
bocytopenia, elevated ESR.
ürìoa|ysìs Peisistent pioteinuiia, casts.
0IACN0SIS
Made on the basis of clinical findings, histopa-
thology, lupus band test, and seiology within
the fiamewoik of the ievised Ameiican Rheu-
matism Association (ARA) ciiteiia foi classifi-
cation of SLE (Table 14-6).
Fk0CN0SIS
Five-yeai suivival is 93%.
MANAC£M£NI
Ceoera| Measures Rest, avoidance of sun
exposuie.
Iodìcatìoos Ior Fredoìsooe (60 mg/d in
divided doses): (1) CNS involvement, (2) ie-
nal involvement, (3) seveiely ill patients with-
out CNS involvement, (4) hemolytic ciisis, (5)
thiombocytopenia.
Coocomìtaot Immuoosuppressìve 0ru¿s Aza-
thiopiine, mycophenolate mofetil, methotiexate,
cyclophosphamide, depending on oigan in-
volvement and activity of disease. In ienal dis-
ease, cyclophosphamide IV bolus theiapy.
Aotìma|arìa|s Hydioxychloioquine is useful
foi tieatment of the skin lesions in subacute and
chionic SLE but does not ieduce the need foi
piednisone. Obseive piecautions in the use of
hydioxychloioquine. Alteinative: chloioquine,
quinaciine.
Iovestì¿atìooa| Ant-TNF agents; efalizumab,
iituximab, leflunomide, anti-inteifeion
agents.
CüIAN£0üS IüFüS £k¥Ih£MAI0SüS
ACüI£ CüIAN£0üS I£
Foi skin lesions and systemic manifestations see
°Systemic Lupus Eiythematosus," above.
IA8I£ 14-5 Patho¿enic Autoantibodies in Systemic Lupus Erythematosus
Aot|geo Spec|I|c|ty Preva|eoce, % Na|o 0||o|ca| £IIects
Au||·dou||e·º||+ud ||A 10-30 K|due, d|ºe+ºe, º||u d|ºe+ºe
|uc|eoºoreº o0-90 K|due, d|ºe+ºe, º||u d|ºe+ºe
ko !0-+0 '||u d|ºe+ºe, ||due, d|ºe+ºe, |e|+| |e+|| p|o||erº
|+ ¹5-20 |e|+| |e+|| p|o||erº
'r ¹0-!0 K|due, d|ºe+ºe
|\|A |ecep|o| !!-50 B|+|u d|ºe+ºe
||oºp|o||p|dº 20-!0 I||or|oº|º, p|eçu+uc, |oºº
·Ac||u|u 20 K|due, d|ºe+ºe
C¹q +0-50 K|due, d|ºe+ºe
|0IE. |\|A, |·re||,|·|·+ºp+||+|e.
'0ukCE. A|||e.|+|ed ||or A k+|r+u, |A |ºeu|e|ç. | Euç| l \ed !53.929, 2003.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 381
â
FICük£ 14-21 Acute SI£ â. ked·|o·.|o|+ceouº, We||·der+|c+|ed p+pu|eº +ud p|+queº ou ||e do|º+ o| ||e
||uçe|º +ud |+udº, c|+|+c|e||º||c+||, ºp+||uç ||e º||u o.e||,|uç ||e jo|u|º. I||º |º +u |rpo||+u| d|||e|eu||+| d|+çuoº||c
º|çu W|eu couº|de||uç de|r+|or,oº|||º, W||c| c|+|+c|e||º||c+||, |u.o|.eº ||e º||u o.e| ||e jo|u|º (corp+|e W|||
||ç. ¹+·¹13 8. |+|r+| e|,||er+ r+|u|, ou ||e ||uçe|||pº. I||º |º p+||oçuorou|c.
8
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 382
IA8I£ 14-6 1982 Revised ARA Criteria for C|assification of Systemic Lupus Erythematosus
*
0r|ter|oo 0eI|o|t|oo
¹. \+|+| |+º| ||\ed e|,||er+, ||+| o| |+|ºed, o.e| ||e r+|+| er|ueuceº, |eud|uç |o ºp+|e
||e u+ºo|+||+| |o|dº.
2. ||ºco|d |+º| E|,||er+|ouº |+|ºed p+|c|eº W||| +d|e|eu| |e|+|o||c ºc+||uç +ud |o|||cu|+|
p|uçç|uç, +||op||c ºc+|||uç r+, occu| |u o|de| |eº|ouº.
!. ||o|oºeuº|||.||, '||u |+º|eº +º + |eºu|| o| uuuºu+| |e+c||ou |o ºuu||ç||, |, p+||eu| ||º|o|, o|
p|,º|c|+u o|ºe|.+||ou.
+. 0|+| u|ce|º 0|+| o| u+ºop|+|,uçe+| u|ce|+||ou, uºu+||, p+|u|eºº, o|ºe|.ed |, +
p|,º|c|+u.
5. A|||||||º |oue|oº|.e +|||||||º |u.o|.|uç |Wo o| ro|e pe||p|e|+| jo|u|º, c|+|+c|e||/ed
|, |eude|ueºº, ºWe|||uç, o| e||uº|ou.
o. 'e|oº|||º +. ||eu||||º-cou.|uc|uç ||º|o|, o| p|eu||||c p+|u o| |u| |e+|d |, + p|,º|c|+u
o| e.|deuce o| p|eu|+| e||uº|ou ¤·
|. |e||c+|d|||º-docureu|ed |, ECC o| |u| o| e.|deuce o| pe||c+|d|+|
e||uº|ou.
1. keu+| d|ºo|de| +. |e|º|º|eu| p|o|e|uu||+-0.5ç/d o| !+ || qu+u|||+||ou uo| pe||o|red ¤·
|. Ce||u|+| c+º|º-r+, |e |ed ce||, |eroç|o||u, ç|+uu|+|, |u|u|+|, o| r|\ed.
3. |eu|o|oç|c d|ºo|de| +. 'e|/u|eº-|u ||e +|ºeuce o| o||eud|uç d|uçº o| |uoWu re|+|o||c
de|+uçereu|º, e.ç., u|er|+, |e|o+c|doº|º, o| e|ec||o|,|e |r|+|+uce ¤·
|. |º,c|oº|º-|u ||e +|ºeuce o| o||eud|uç d|uçº o| |uoWu re|+|o||c
de|+uçereu|º, e.ç., u|er|+, |e|o+c|doº|º, o| e|ec||o|,|e |r|+|+uce.
9. ner+|o|oç|c d|ºo|de| +. nero|,||c +uer|+-W||| |e||cu|oc,|oº|º ¤·
|. |eu|opeu|+- ·+000/µ| |o|+| ou |Wo o| ro|e occ+º|ouº ¤·
c. |,rp|opeu|+- ·¹500/µ| ou |Wo o| ro|e occ+º|ouº ¤·
d. I||or|oc,|opeu|+- ·¹00,000/µ| |u ||e +|ºeuce o| o||eud|uç d|uçº.
¹0. |rruuo|oç|c d|ºo|de| +. Au||·||A-+u|||od, |o u+||.e ||A |u +|uo|r+| |||e| ¤·
|. Au||·'r-p|eºeuce o| +u|||od, |o 'r uuc|e+| +u||çeu ¤·
c. |oº|||.e ||ud|uç o| +u||p|oºp|o||p|d +u|||od|eº |+ºed ou (¹) +u
+|uo|r+| ºe|ur |e.e| o| |çC o| |ç\ +u||c+|d|o||p|u +u|||od|eº, (2) +
poº|||.e |eº| |eºu|| |o| |upuº +u||co+çu|+u| uº|uç + º|+ud+|d re||od,
o| (!) + |+|ºe·poº|||.e ºe|o|oç|c |eº| |o| º,p||||º |uoWu |o |e poº|||.e
|o| +| |e+º| o rou||º +ud cou|||red |, ueç+||.e í·-¤¤¤-¤c ¤c||·!±¤
|rro||||/+||ou o| ||uo|eºceu| ||epouer+| +u|||od, +|ºo|p||ou |eº|.
¹¹. Au||uuc|e+| +u|||od, Au +|uo|r+| |||e| o| +u||uuc|e+| +u|||od, |, |rruuo||uo|eºceuce o| +u
equ|.+|eu| +ºº+, +| +u, po|u| |u ||re +ud |u ||e +|ºeuce o| d|uçº
|uoWu |o |e +ººoc|+|ed W||| 'd|uç·|uduced |upuº¨ º,ud|ore.
*I|e p|opoºed c|+ºº|||c+||ou |º |+ºed ou ¹¹ c|||e||+. |o| ||e pu|poºe o| |deu|||,|uç p+||eu|º |u c||u|c+| º|ud|eº, + pe|ºou º|+|| |e º+|d |o |+.e '|E ||
+u, + o| ro|e o| ||e ¹¹ c|||e||+ +|e p|eºeu|, ºe||+||, o| º|ru||+ueouº|,, du||uç +u, |u|e|.+| o| o|ºe|.+||ou.
'0ukCE. kep||u|ed ||or E\ I+u e| +|. A|||||||º k|eur 25.¹21¹, ¹932. uºed |, pe|r|ºº|ou o| ||e Are||c+u Co||eçe o| k|eur+|o|oç,.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 383
£FI0£MI0I0C¥
A¿e oI 0oset Young and middle age.
kace Uncommon in blacks oi Hispanics.
Sex Females > males.
Iocìdeoce About 10% of the LE population.
Frecìpìtatìo¿ Factors Sunlight exposuie.
CIINICAI MANIF£SIAII0N
Rathei sudden onset with annulai oi pso-
iiasifoim plaques eiupting on the uppei tiunk,
aims, doisa of the hands, usually aftei exposuie
to sunlight; mild fatigue, malaise; some aithial-
gia, fevei of unknown oiigin.
'||u |eº|ouº o| 'C|E +|e +uuu|+| o| pºo||+º||o|r
(||ç. ¹+·22).
|+||eu|º W||| 'C|E r+, |+.e ºore o| ||e c|||e||+
o| '|E +º de||ued |, ||e AkA, |uc|ud|uç p|o·
|oºeuº|||.||,, +||||+|ç|+º, ºe|oº|||º, |eu+| d|ºe+ºe,
+ud ºe|o|oç|c +|uo|r+||||eº. 50° o| p+||eu|º W|||
'C|E |+.e '|E.
||+c||c+||, +|| |+.e +u||·ko (''·A) +ud roº| |+.e
+u||·|+ (''·B) +u|||od|eº. I|e ºe||ouº o|ç+u
|u.o|.ereu| o| '|E |º uucorrou.
I|e c||u|c+| º||u |eº|ouº +|e ||e d|º||uc||.e |e+|u|e
o| 'C|E.
Sü8ACüI£ CüIAN£0üS IüFüS £k¥Ih£MAI0SüS (SCI£)
Skìo Iesìoos Two ìy¡es : (1) Psoríasí[orm ¡a¡u-
|osquamous , shaiply defined, with slight delicate
scaling (Fig. 14-22), evolving into biight ied
confluent plaques that aie oval, aicifoim, oi
polycyclic, just as in psoiiasis; and (2) annu|ar ,
biight ied annulai lesions with cential iegies-
sion and little scaling. In both theie may be
telangiectasia, but theie is no folliculai plug-
ging and less induiation than in CCLE. Lesions
iesolve with slight atiophy (no scaiiing) and
hypopigmentation.
DIstrIhutIvn Scatteied, disseminated in light-
exposed aieas: shouldeis, extensoi suiface of
the aims, doisal suiface of the hands, uppei
back, V-neck aiea of the uppei chest.
FICük£ 14-22 Subacute cutaoeous |upus erythematosus w|de|, ºc+||e|ed, e|,||er+|ouº·|o·.|o|+ceouº,
ºc+||uç, We||·der+|c+|ed p|+queº ou ||e ||uu|, uec|, +ud +|rº, r|r|c||uç ||e c||u|c+| +ppe+|+uce o| pºo||+º|º .u|ç+||º.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 384
0ther Iesìoos Peiiungual telangiectasia, dif-
fuse nonscaiiing alopecia.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y aod Immuoopatho|o¿y As
in ACLE, LBT positive in 60%.
üv Iestìo¿ Most patients have a lowei than
noimal UVB minimum eiythema dose (MED).
Typical SCLE lesions may develop in UVB test
sites.
Sero|o¿y ANA piesent in 60-80%. Antibod-
ies to Ro(SS-A) positive in > 80%, to La(SS-B)
in 30-50%; high levels of ciiculating immune
complexes.
0ther Iaboratory Iests Patients with SCLE,
paiticulaily those with manifest systemic
involvement, may have a numbei of laboia-
toiy abnoimalities, including anemia, leukope-
nia, lymphopenia, hematuiia, pioteinuiia, and
depiessed complement levels.
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical findings confiimed by histology and
immunopathology. The extensive involvement
is fai moie than is evei seen in CCLE, and the
distinctive eiuption is a maikei foi SCLE.
0ìIIereotìa| 0ìa¿oosìs Red plaques of dei-
matomyositis, secondaiy syphilis, psoiiasis, seb-
oiiheic deimatitis, tinea coipoiis, polymoiphic
light eiuption.
C0ükS£ AN0 Fk0CN0SIS
A bettei piognosis than foi SLE in geneial.
Some patients with ienal (and CNS) involve-
ment have a guaided piognosis. The skin lesions
can disappeai completely, but occasionally, a
vitiligo-like depigmentation iemains foi some
months. Women with Ro(SS-A)-positive SCLE
may give biith to babies with neonatal lupus
and congenital heait block.
MANAC£M£NI
Iopìca| Anti-inflammatoiy glucocoiticoids
and topical pimeciolimus and taciolimus aie
only paitially helpful.
Systemìc Systemic tieatment usually iequiied.
T|a|íJomíJe (100-300 mg/d) is veiy effective foi
skin lesions but not foi systemic involvement.
HyJroxyt||oroquíne , 400 mg/d; if this does not
contiol the skin lesions, quinaciine hydiochlo-
iide, 100 mg/d, can be added. The bizaiie yellow
skin coloi caused by quinaciine can be some-
what modified by -caiotene, 60 mg tid.
I||º c||ou|c, |udo|eu| º||u d|ºe+ºe |º c|+|+c|e||/ed
|, º|+|p|, r+|ç|u+|ed, ºc+|,, |u|||||+|ed, +ud |+|e|
+||op||c |ed ('d|ºco|d¨) p|+queº, uºu+||, occu|||uç
ou |+|||u+||, e\poºed +|e+º (||çº. ¹+·2!, ¹+·2+).
I||º d|ºo|de|, |u roº| c+ºeº, |º pu|e|, cu|+ueouº
W|||ou| º,º|er|c |u.o|.ereu| (|r+çe ¹+·!).
noWe.e|, CC|E |eº|ouº r+, occu| |u '|E.
CC|E r+, r+u||eº| +º c||ou|c d|ºco|d |E (C||E,
ºee |e|oW) o| |E p+uu|cu||||º (I+||e ¹+·+).
Chk0NIC CüIAN£0üS IüFüS £k¥Ih£MAI0SüS (CCI£) |C|·9 . o95.+
°
|C|·¹0 . |9!.0
CIASSIC Chk0NIC 0ISC0I0 I£ (C0I£)
£FI0£MI0I0C¥
A¿e oI 0oset 20-45 yeais.
Sex Females > males.
kace Possibly moie seveie in blacks.
CIINICAI MANIF£SIAII0N
Can be piecipitated by sunlight but to a
lessei extent than ACLE oi SCLE. Lesions last
foi months to yeais. Usually no symptoms,
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 385
FICük£ 14-23 Chrooìc cutaoeous |upus erythematosus we||·der+|c+|ed, e|,||er+|ouº, |,pe||e|+|o||c
p|+queº W||| +||op|,, |o|||cu|+| p|uçç|uç, +ud +d|e|eu| ºc+|e ou |o|| c|ee|º. I||º |º ||e c|+ºº|c p|eºeu|+||ou o|
c||ou|c d|ºco|d |E.
FICük£ 14-24 Chrooìc cutaoeous |upus
erythematosus: scarrìo¿ I|e|e +|e ru|||p|e
ºc+||ed p|+queº ||+| |+.e + dep|eººed ceu|e|
+ud +u +c||.e, º|||| e|,||er+|ouº +ud ºc+|, r+|·
ç|u |u ||e |+ce o| |||º o0·,e+|·o|d |er+|e |+|re|.
'c+|||uç |+º |ed |o couº|de|+||e d|º||çu|ereu|.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 386
sometimes slightly piuiitic oi smaiting. No
geneial symptoms.
Skìo Iesìoos Biight ied papules evolving
into plaques, shaiply maiginated, with adhei-
ent scaling (Fig. 14-23). Scales aie difficult to
iemove and show spines on the undeisuiface
(magnifying lens) iesembling caipet tacks.
Plaques aie iound oi oval, annulai oi polycy-
clic, with iiiegulai boideis and expand in the
peiipheiy and iegiess in the centei, iesulting
in depiession of lesions, atiophy, and eventu-
ally scaiiing (Fig. 14-24). Folliculai plugging
and dilated follicles may peisist in atiophic le-
sions but eventually disappeai so that smooth,
whitish scais iesult that aie paitially sui-
iounded by a still active inflammatoiy and
iaised boidei (Fig. 14-24). °Buined out" le-
sions may be pink oi white (hypomelanosis)
macules and scais (Fig. 14-26), but scaiied
lesions may also show hypeipigmentation,
especially in peisons with biown oi black skin
(Fig. 14-25).
DIstrIhutIvn und SItes v] PredI|ectIvn CDLE
may be localized oi geneialized, occuiiing pie-
dominantly on the face and scalp; doisa of foie-
aims, hands, fingeis, toes, and less fiequently,
the tiunk (Image 14-4) (Fig. 33-32).
Sca|p Scaiiing alopecia with iesidual inflam-
mation and folliculai plugging (Fig. 14-26; see
Section 32, Figs. 32-15, 32-16).
Mucous Membraoes < 5% of patients have lip
involvement (hypeikeiatosis, hypeimelanotic
scaiiing, eiythema) and atiophic eiythematous
oi whitish aieas with oi without ulceiation on
the buccal mucosa, tongue, and palate. Naí|
a¡¡araìus Nail dystiophy if nail matiix is in-
volved.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Hypeikeiatosis, atiophy
of the epideimis, folliculai plugging, lique-
faction degeneiation of the basal cell layei.
Edema, dilatation of small blood vessels,
and peiifolliculai and peiiappendageal lym-
phocytic inflammatoiy infiltiate. Stiong PAS
ieaction of the subepideimal, thickened base-
ment zone.
ImmuooI|uoresceoce LBT positive in 90% of
active lesions at least 6 weeks old and not
iecently tieated with topical glucocoiticoids.
LBT negative in buined-out (scaiied) lesions
and in the noimal skin, both sun-exposed and
nonexposed.
Sero|o¿y Low incidence of ANA with titeis
>1:16.
hemato|o¿y Occasionally leukopenia (<4500/
L).
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical findings confiimed by histopathology
and immunopathology. The discoid lesions
of CDLE may closely mimic atìínít |eraìosís .
P|aque ¡soríasís and scaling discoid LE without
atiophy and scaiiing may be difficult to dis-
tinguish, especially on the doisa of the hands;
histopathology peimits distinction. Po|ymor-
¡|ous |íg|ì eru¡ìíon LE (PMLE) may pose a
pioblem. PMLE does not develop atiophy oi
folliculai plugging, and does not occui in unex-
posed aieas-mouth, haiiy scalp. Lít|en ¡|anus
can be confusing, but the biopsy is distinctive.
Howevei, theie is a lichen planus-LE, syndiome
oveilap of featuies. Lu¡us ·u|garís and ìínea
[atía|ís.
C0ükS£ AN0 Fk0CN0SIS
Only 1-5% may develop SLE; with localized le-
sions, complete iemission occuis in 50%; with
geneialized lesions, iemissions aie less fiequent
(<10%). Noìe agaín : CCLE lesions may be the
piesenting cutaneous sign of SLE.
MANAC£M£NI
Freveotìoo Topical sunscieens (SPF > 30) iou-
tinely.
Ioca| C|ucocortìcoìds aod Ca|cìoeurìo Iohìbì-
tors Usually not veiy effective; topical fluoii-
nated glucocoiticoids with caution because of
atiophy. Intialesional tiiamcinolone acetonide,
3-5 mg/mL, foi small lesions.
Aotìma|arìa|s Hydioxychloioqine, 6.5 mg/kg
body weight pei day. If hydioxychloioquine is
ineffective, add quinaciine, 100 mg thiee times
a day. Monitoi foi oculai side effects.
ketìooìds Hypeikeiatotic CDLE lesions ie-
spond well to systemic acitietin (0.5 mg/kg
body weight).
Iha|ìdomìde 100-300 mg/d is effective. Ob-
seive contiaindications.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 38T
FICük£ 14-25 Chrooìc cutaoeous |upus erythematosus: hyperpì¿meotatìoo Aº |u||+rr+|o|, |eº|ouº
|eºo|.e ||e|e r+, |e |,pe|p|çreu|+||ou o| ||e +||op||c +ud p+|||+||, ºc+||ed |eº|ou+| º||u, p+|||cu|+||, |u '|I |||
+ud |\ p+||eu|º. A|||ouç| ||e º||u |eº|ouº We|e CC|E, ||e p+||eu| |+d '|E.
FICük£ 14-26 Chrooìc cutaoeous |upus erythematosus |u.o|.ereu| o| ||e ºc+|p |+º |ed |o corp|e|e
|+|| |oºº W||| |eº|du+| e|,||er+, +||op|,, +ud W|||e ºc+|||uç |u |||º ||+c| r+|e. '|+|p der+|c+||ou o| ||e |eº|ouº
|u ||e pe||p|e|, |ud|c+|eº ||+| ||eºe |eº|ouº o||ç|u+||, We|e C||E p|+queº.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 388
CIINICAI MANIF£SIAII0N
May piecede oi follow the onset of discoid
lesions by seveial yeais. Nodules aie asympto-
matic, tendei, oi sometimes painful.
Skìo Iesìoos Deep-seated nodules oi plate-
like infiltiations with oi without giossly vis-
ible epideimal changes oi change of coloi;
indolent and fiim, sometimes tendei oi pain-
ful, and aie bettei felt than seen. The oveily-
ing skin may be noimal, eiythematous, oi
biownish oi exhibit typical lesions of CDLE.
Lesions evolve into deep depiessions (Fig. 14-
27) but may also ulceiate; in this case theie is
scaiiing.
DIstrIhutIvn Scalp, face, uppei aims (Fig.
14-27), tiunk (especially the bieasts), thighs,
and buttocks.
Systems kevìew Mild SLE may be piesent
(35%).
C||ou|c |upuº p+uu|cu||||º |º + |o|r o| CC|E |u
W||c| ||e|e +|e |||r, c||curºc|||ed ºu|cu|+ueouº
uodu|eº.
|e+d |o ºu|cu|+ueouº +||op|, +ud ºc+|||uç |eºu||·
|uç |u ºuu|eu +|e+º.
'u|cu|+ueouº uodu|eº occu| |o|| W||| +ud W|||·
ou| ||E |eº|ouº o| o.e||,|uç º||u.
uºu+||, + |o|r o| cu|+ueouº |upuº, |u| !5° o|
p+||eu|º |+.e r||d '|E (ºee |r+çe ¹+·!).
',¤¤¤,¤. |upuº e|,||er+|oºuº p|o|uuduº.
Chk0NIC IüFüS FANNICüIIIIS
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Subcutaneous layei.
Neciobiosis with fibiinoid deposits, dense lym-
phocytic infiltiates, and vasculitis; latei, hyalini-
zation of the fat lobules; fibiosis; theie may be
consideiable mucinous deposits.
0ther In patients with SLE theie aie typical
hematologic and seiologic abnoimalities.
0IFF£k£NIIAI 0IACN0SIS
Moiphea, eiythema nodosum, saicoid, miscel-
laneous types of panniculitis.
MANAC£M£NI
· Antimalaiials
· Thalidomide as foi othei foims of cutnaeous
LE. Bewaie of contiaindications.
· Systemic glucocoiticoids
(shoit couise)
FICük£ 14-2T Iupus paooìc-
u|ìtìs C||ou|c p+uu|cu||||º W|||
+||op|, o| ||e ºu|cu|+ueouº ||ººue,
|eºu|||uç |u |+|çe ºuu|eu +|e+º o|
o.e||,|uç º||u, |ep|eºeu||uç |eºo|.·
|uç |eº|ouº. w|e|e e|,||er+ |º
º|||| .|º|||e, p+|p+||ou |e.e+|º |||r
ºu|cu|+ueouº uodu|eº +ud p|+queº.
A|ºo, ºore |eº|ouº |e.e+| ºc+|||uç |u
||e ceu|e|.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 389
£FI0£MI0I0C¥
Freva|eoce 20 pei million of U.S. population.
A¿e oI 0oset 30-50 yeais.
Sex Female:male iatio, 4:1.
CIASSIFICAII0N
Systemic scleiodeima can be divided into two
subsets: |ímíìeJ sysìemít st|eroJerma (lSSc) and
Jí[[use sysìemít st|eroJerma (dSSc). lSSc patients
compiise 60%; patients aie usually female;
oldei than those with dSSc; and have a long
histoiy of Raynaud phenomenon with skin
involvement limited to hands, feet, face, and
foieaims (acioscleiosis) and a high incidence
of anticentiomeiic antibodies. lSSc includes the
CREST syndiome, and systemic involvement
may not appeai foi yeais; patients usually die
of othei causes. dSSc patients have a ielatively
iapid onset and diffuse involvement, not only
of hands and feet but also of the tiunk and face,
synovitis, tendosynovitis, and eaily onset of in-
teinal involvement. Anticentiomeie antibodies
aie uncommon, but Scl-70 (antitopoisomeiase
I) antibodies aie piesent in 33%.
£II0I0C¥ AN0 FAIh0C£N£SIS
Unknown. Piimaiy event might be endothelial
cell injuiy in blood vessels, the cause of which is
unknown. Eaily in couise, taiget oigan edema
occuis, followed by fibiosis; cutaneous capillaiies
aie ieduced in numbei; iemaindei dilate and pio-
lifeiate, becoming visible telangiectasia. Fibiosis
due to oveipioduction of collagen by fibioblasts.
CIINICAI MANIF£SIAII0N
Raynaud phenomenon (see p. 394) with digital
pain, coldness. Pain/stiffness of fingeis, knees.
'c|e|ode|r+ |º + uo| ºo |+|e ru|||º,º|er d|ºo|·
de| c|+|+c|e||/ed |, |u||+rr+|o|,, .+ºcu|+|, +ud
ºc|e|o||c c|+uçeº o| ||e º||u +ud .+||ouº |u|e|u+|
o|ç+uº, eºpec|+||, ||e |uuçº, |e+||, +ud C| ||+c|.
||r||ed º,º|er|c ºc|e|ode|r+ (|''c) (o0°) +ud
d|||uºe º,º|er|c ºc|e|ode|r+ (d''c) +|e |ecoç·
u|/ed.
C||u|c+| |e+|u|eº +|W+,º p|eºeu| +|e º||u ºc|e|oº|º
+ud k+,u+ud p|euoreuou.
Couº|de|+||e ro|||d||,, ||ç| ro||+|||, |o| d''c
',¤¤¤,¤º . ||oç|eºº|.e º,º|er|c ºc|e|oº|º, º,º·
|er|c ºc|e|oº|º, º,º|er|c ºc|e|ode|r+.
SCI£k00£kMA |C|·9 . 1¹0.¹
°
|C|·¹0 . \!+
Migiatoiy polyaithiitis. Heaitbuin, dysphagia,
especially with solid foods. Constipation, di-
aiihea, abdominal bloating, malabsoiption,
weight loss. Exeitional dyspnea, diy cough.
Skìo Hunds/Feet Ear|y : Raynaud phenom-
enon with tiiphasic coloi changes, i.e., palloi,
cyanosis, iuboi (Fig. 14-28B, see also Fig. 14-
32). Piecedes scleiosis by months and yeais.
Nonpitting edema of hands/feet. Painful ul-
ceiations at fingeitips (°iat bite neciosis") (Fig.
14-29À), knuckles; heal with pitted scais. Laìe :
scleiodactyly with tapeiing of fingeis (ma-
donna fingeis) (Fig. 14-28À) with waxy, shiny,
haidened skin, which is tightly bound down
and does not peimit folding oi wiinkling; leath-
eiy ciepitation ovei joints, flexion contiactuies;
peiiungual telangiectasia, nails giow clawlike
ovei shoitened distal phalanges (Fig. 14-28B).
Bony iesoiption and ulceiation iesults in loss
of distal phalanges.
As scleiosis pioceeds pioximally, theie aie
loss of sweat glands with anhidiosis and thin-
ning and complete loss of haii on distal ex-
tiemities.
Fuce Ear|y : peiioibital edema. Laìe : edema
and fibiosis iesult in loss of noimal facial lines,
masklike (patients look youngei than they aie)
(Fig. 14-30), thinning of lips, miciostomia, ia-
dial peiioial fuiiowing (Fig. 14-29B), beak-like
shaip nose. Telangiectasia (Fig. 14-31) and dif-
fuse hypeipigmentation.
Trun| In dSSc the chest and pioximal uppei
and lowei extiemities aie involved eaily. Tense,
stiff, and waxy appeaiing skin that cannot be
folded. Impaiiment of iespiiatoiy movement of
chest wall and of joint mobility.
0ther Chao¿es Cutunevus Cu|cI]IcutIvn
Occuis on fingeitips oi ovei bony piominences
oi any scleiodeimatous site; may ulceiate and
exude white paste.
Cv|vr Chunges Hypeipigmentation that may
be geneialized and on the extiemities may be
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 390
accompanied by peiifolliculai hypopigmenta-
tion.
Mucvus Memhrunes Scleiosis of sublingual
ligament; uncommonly, painful induiation of
gums, tongue.
DIstrIhutIvn v] LesIvns Ear|y : in lSSc eaily
involvement is seen on fingeis, hands, and face,
and in many patients scleiodeima iemains
confined to these iegions. Laìe : the distal uppei
and lowei extiemities may be involved and oc-
casionally the tiunk. In dSSc scleiosis of the ex-
tiemities and the tiunk may stait soon oi soon
aftei oi concomitant with acial involvement.
C|ìoìca| varìaot CREST syndiome, i.e.,
t alcinosis cutis - R aynaud phenomenon -
e sophageal dysfunction - s cleiodactyly - ì el-
angiectasia. Maculai, matlike telangiectasia, es-
pecially the face (Fig. 14-31), uppei tiunk, and
hands; also in the entiie GI tiact. Calcinosis
ovei bony piominences, fingeitips, elbows, and
tiochanteiic iegions.
C£N£kAI £XAMINAII0N
£sopha¿us Dysphagia, diminished peiistalsis,
ieflux esophagitis.
Castroìotestìoa| System Small intestine in-
volvement may pioduce constipation, diaiihea,
bloating, and malabsoiption.
Iuo¿ Pulmonaiy fibiosis and alveolitis.
Reduction of pulmonaiy function due to ie-
stiicted movement of chest wall.
heart Caidiac conduction defects, heait fail-
uie, peiicaiditis.
kìdoey Renal involvement occuis in 45%.
Slowly piogiessive uiemia, malignant hypei-
tension.
Muscu|oske|eta| System Caipal tunnel syn-
diome. Muscle weakness.
IA80kAI0k¥ £XAMINAII0NS
0ermatopatho|o¿y Ear|y : mild cellulai infil-
tiate aiound deimal blood vessels, ecciine coils,
and at the deimal subcutaneous inteiphase.
Laìe : bioadening and homogenization of col-
lagen bundles, obliteiation and deciease of
inteibundle spaces, thickening of deimis with
ieplacement of uppei oi total subcutaneous fat
by hyalinized collagen. Paucity of blood vessels,
thickening/hyalinization of vessel walls.
Autoaotìbodìes Patients with dSSc have cii-
culating autoantibodies by ANA testing. Au-
toantibodies ieact with centiomeie pioteins
oi DNA topoisomeiase I; fewei patients have
antinucleolai antibodies. Anticentiomeiic au-
toantibodies occui in 21% of dSSc and 71% of
CREST patients, DNA topoisomeiase I (Scl-70)
antibodies in 33% of dSSc and 18% of CREST
patients.
FICük£ 14-28 Sc|eroderma (|SSc): acrosc|erosìs â. n+udº +ud ||uçe|º +|e eder+|ouº (uoup||||uç), º||u
|º W|||ou| º||u |o|dº +ud |ouud doWu. ||º|+| ||uçe|º +|e |+pe|ed (r+douu+ ||uçe|º) 8. ||uçe|º º|oW |o||
||u|º| e|,||er+ +ud .+ºocouº|||c||ou (||ue +ud W|||e). k+,u+ud p|euoreuou. ||uçe|º +|e eder+|ouº, ||e º||u |º
|ouud doWu. ||º|+| p|+|+uçeº (|ude\ +ud ||||d ||uçe|) +|e º|o||eued, W||c| |º +ººoc|+|ed W||| |ou, |eºo|p||ou.
â 8
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 391
0IACN0SIS AN0 0IFF£k£NIIAI 0IACN0SIS
Clinical findings confiimed by deimatopathol-
ogy.
0ìIIereotìa| 0ìa¿oosìs Dí[[use st|erosís : mixed
connective tissue disease, eosinophilic fascii-
tis, scleiomyxedema, moiphea, poiphyiia cu-
tanea taida, chionic giaft-veisus-host disease
(GVHD), lichen scleiosus et atiophicus, poly-
vinyl chloiide exposuie, adveise diug ieaction
(pentazocine, bleomycin). Gadolinium and ne-
phiogenic systemic fibiosis (see Section 17).
C0ükS£ AN0 Fk0CN0SIS
Couise chaiacteiized by slow, ielentless pio-
giession of skin and/oi visceial scleiosis; the
10-yeai suivival iate is >50%. Renal disease
is the leading cause of death, followed by
caidiac and pulmonaiy involvement. Spon-
taneous iemissions do occui. lSSc, which
includes the CREST syndiome, piogiesses
moie slowly and has a moie favoiable piog-
nosis; some cases do not develop visceial
involvement.
â
FICük£ 14-29 Sc|eroderma (|SSc): acrosc|erosìs â. I,p|c+| '|+| |||e¨ uec|oºeº +ud u|ce|+||ouº o|
||uçe|||pº. 8. I||uu|uç o| ||pº-r|c|oº|or|+ (W||c| Wou|d º|oW |e||e| W|eu p+||eu| +||erp|º |o opeu |e|
rou||), |+d|+| pe||o|+| |u||oW|uç. Be+||||e º|+|p uoºe.
8
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 392
MANAC£M£NI
Systemic glucocoiticoids may be of benefit
foi limited peiiods eaily in the disease. All
othei systemic tieatments (EDTA, aminocap-
ioic acid, D-penicillamine, ¡ara -aminoben-
zoate, colchicine) have not been shown to be
of lasting benefit. Immunosuppiessive diugs
(cyclospoiine, methotiexate, cyclophospha-
mide, mycophenolate mofetil) have shown
impiovement of skin scoie but only limited
benefit foi systemic involvement. Photophei-
esis: impiovement in one thiid of patients. Im-
munoablation/stem cell tiansplantation and
oial toleiization to type I collagen: ongoing
studies.
FICük£ 14-30 Sc|eroderma (dSSc) \+º||||e |+c|eº W||| º||e|c|ed, º||u, º||u +ud |oºº o| uo|r+| |+c|+| ||ueº
ç|.|uç + ,ouuçe| +ppe+|+uce ||+u +c|u+| +çe, ||e |+|| |º d,ed. I||uu|uç o| ||e ||pº +ud pe||o|+| ºc|e|oº|º |eºu|| |u +
ºr+|| rou||. 'c|e|oº|º (W||||º|, ç||º|eu|uç +|e+º) +ud ru|||p|e |e|+uç|ec|+ºeº (uo| .|º|||e +| |||º r+çu|||c+||ou) +|e
+|ºo p|eºeu|.
S£CII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 393
A d''c·|||e coud|||ou occu|º |u pe|ºouº e\poºed
|o po|,.|u,| c||o||de.
B|eor,c|u +|ºo p|oduceº pu|rou+|, ||||oº|º +ud
k+,u+ud p|euoreuou |u| uo| º||u ºc|e|oº|º.
Cu|+ueouº c|+uçeº |ud|º||uçu|º|+||e ||or d''c·
|||e ºc|e|oº|º o| º||u, +ccorp+u|ed |, r,+|ç|+,
pueurou|||º, r,oc+|d|||º, ueu|op+||,, +ud eu·
cep|+|op+||,, +|e |e|+|ed |o ||e |uçeº||ou o|
ce||+|u |o|º o| | ·||,p|op|+u ( -¤º·¤¤¤|·|·c·¤,c|¸·c
º,¤!·¤¤- ),
I|e |¤··: ¤·| º,¤!·¤¤- ||+| occu||ed |u +u
ep|der|c |u 'p+|u |u ¹93¹ +||ec||uç 25,000 peop|e
W+º due |o ||e couºurp||ou o| deu+|u|ed |+pe
ºeed o||. A||e| +u +cu|e p|+ºe, W||| |+º|, |e·
.e|, pueurou|||º, +ud r,+|ç|+, ||e º,ud|ore
p|oç|eººeº |o + coud|||ou W||| ueu|oruºcu|+|
+|uo|r+||||eº +ud ºc|e|ode|r+·|||e º||u |eº|ou